Inguinal lymph nodes agenesia in a patient with Schimmelpenning-Feuerstein-Mims syndrome with proven somatic KRAS mutation.

Schimmelpenning-Feuerstein-Mims (SFM) syndrome is a neurocutaneous disorder that can affect many body systems. The principal and most characteristic anomalies are craniofacial naevus sebaceous in association with neurological, ocular and skeletal findings. The presence of vascular malformations in this condition is unusual; nevertheless, vascular malformations have been suggested by many authors to be part of the spectrum of the same disease. Few cases have been published on the association of SFM with lymphatic malformations. This syndrome is categorized as a mosaic RASopathy due to postzygotic mutations in the HRAS, KRAS or NRAS genes. These genes are involved in the RAF-MEK-ERK signalling pathway, which is activated by mutant cells, increasing cellular proliferation. These mutations have been found only in naevus sebaceous cells, and may be also the explanation for many of the associated pathologies. We report a case of an 18-year-old boy diagnosed with SFM syndrome associated with lymphatic malformation in the legs and agenesia of the inguinal lymph nodes. The lymphatic alterations were diagnosed by gammography of the legs. The genetic diagnosis was confirmed by the presence of a KRAS postzygotic mutation in naevus sebaceous cells of a skin specimen. Genetically confirmed cases of mosaic RASopathies should be used to more accurately characterize phenotypic presentations of this syndrome and develop a future therapeutic strategy, such as molecular targeted therapy.

[Parent controled analgesia system in postoperative pain management in patients undergoing lip and cleft palate surgery]. / Analgesia mediante bomba de infusión continua controlada por el paciente para el manejo postoperatorio del dolor en pacientes intervenidos de fisura palatina y labio fisurado.

OBJECTIVES: Craniofacial clefts surgery associates a painful postoperative pain whose management is complicated with conventional analgesia. PATIENTS AND METHODS: A parent controlled analgesia system was implanted with a continuous perfusion of tramadol, ondansetron and metamizole adjusted by weight. Parents are allowed to administer additional boluses if they observe irritability. We compared the variables of the cleft patients operated before and after the implantation of the system in our center. RESULTS: During 2016, 16 craniofacial clefts were operated (4 cheilorhinoplasties and 12 palatal clefts). No PCA (parent controlled analgesia) system was used. The average time of stay in PICU was 1.5 days. It took an average of 2.5 days to initiate tolerance. The mean of VAS (Visual Analogic Scale) was 3. 53% required major opioids (morphine, fentanyl) not being sufficient analgesia every 3 hours. During 2017, 7 palatal fissures and 4 cheilorhinoplasties were operated (11). Both of them were controlled by PCA. Patients with palatal cleft were admitted to the PICU with a total mean of 0.5 days. The beginning of tolerance was advanced to the first postoperative day. The VAS diminished to 0.5. Only one patient required opioids. 72% did not need to associate any type of analgesia. CONCLUSIONS: The PCA system is a safe and risk-free insurance for analgesia of fissured patients with benefits such as: decrease in pain, stay in PICU, the need for analgesia and initiation of early tolerance.

OBJETIVOS: La cirugía de las fisuras craneofaciales asocia un intenso dolor postoperatorio cuyo manejo resulta complicado con la analgesia convencional. MATERIAL Y METODOS: Utilizamos una bomba de analgesia controlada por los padres que contiene una perfusión continua de tramadol, ondansetrón y metamizol ajustada por peso. Se permite a los padres administrar bolos adicionales si observan irritabilidad. Comparamos variables de los pacientes fisurados intervenidos antes y después de la implantación del sistema en nuestro centro. RESULTADOS: Durante 2016 fueron intervenidos 16 fisurados (4 queilorrinoplastias y 12 fisuras palatinas). En ninguno se empleó bomba de analgesia. El tiempo medio de estancia en UCIP fue 1,5 días. Tardaron de media 2,5 días en iniciar tolerancia. La media de EVA (Escala Analógica Visual) fue de 3. El 53% precisaron opiáceos mayores (morfina, fentanilo), no siendo suficiente la analgesia c/3 horas. Durante 2017 se operaron 7 fisuras palatinas y 4 queilorrinoplastias (11). En todos empleamos bomba. Únicamente ingresaron en UCIP las fisuras palatinas (debido al manejo de la vía aérea) con una media total de 0,5 días. Se adelantó el inicio de tolerancia al primer día postoperatorio. La EVA disminuyo a 0,5. Solo un paciente precisó opiáceos. El 72% no precisó asociar ningún tipo de analgesia. CONCLUSIONES: La bomba de PCA (analgesia controlada por el paciente/por los padres) es un método seguro y exento de riesgo para la analgesia de los pacientes fisurados con beneficios como: disminución del dolor, de la estancia en UCIP, de la necesidad de analgesia e inicio de tolerancia precoz.

Time for radioimmunotherapy: an overview to bring improvements in clinical practice.

Harnessing the patient's own immune system against an established cancer has proven to be a successful strategy. Within the last years, several antibodies blocking critical "checkpoints" that control the activation of T cells, the immune cells able to kill cancer cells, have been approved for the use in patients with different tumours. Unfortunately, these cases remain a minority. Over the last years, radiotherapy has been reported as a means to turn a patient's own tumour into an in situ vaccine and generate anti-tumour T cells in patients who lack sufficient anti-tumour immunity. Indeed, review data show that the strategy of blocking multiple selected immune inhibitory targets in combination with radiotherapy has the potential to unleash powerful anti-tumour responses and improve the outcome of metastatic solid tumours. Here, we review the principal tumours where research in this field has led to new knowledge and where radioimmunotherapy becomes a reality.

Analgesia mediante bomba de infusión continua controlada por el paciente para el manejo postoperatorio del dolor en pacientes intervenidos de fisura palatina y labio fisurado / Parent controled analgesia system in postoperative pain management in patients undergoing lip and cleft palate surgery

Objetivos: La cirugía de las fisuras craneofaciales asocia un intenso dolor postoperatorio cuyo manejo resulta complicado con la analgesia convencional. Material y métodos: Utilizamos una bomba de analgesia controlada por los padres que contiene una perfusión continua de tramadol, ondansetrón y metamizol ajustada por peso. Se permite a los padres administrar bolos adicionales si observan irritabilidad. Comparamos variables de los pacientes fisurados intervenidos antes y después de la implantación del sistema en nuestro centro. Resultados: Durante 2016 fueron intervenidos 16 fisurados (4 queilorrinoplastias y 12 fisuras palatinas). En ninguno se empleó bomba de analgesia. El tiempo medio de estancia en UCIP fue 1,5 días. Tardaron de media 2,5 días en iniciar tolerancia. La media de EVA (Escala Analógica Visual) fue de 3. El 53% precisaron opiáceos mayores (morfina, fentanilo), no siendo suficiente la analgesia c/3 horas. Durante 2017 se operaron 7 fisuras palatinas y 4 queilorrinoplastias (11). En todos empleamos bomba. Únicamente ingresaron en UCIP las fisuras palatinas (debido al manejo de la vía aérea) con una media total de 0,5 días. Se adelantó el inicio de tolerancia al primer día postoperatorio. La EVA disminuyo a 0,5. Solo un paciente precisó opiáceos. El 72% no precisó asociar ningún tipo de analgesia. Conclusiones: La bomba de PCA (analgesia controlada por el paciente/por los padres) es un método seguro y exento de riesgo para la analgesia de los pacientes fisurados con beneficios como: disminución del dolor, de la estancia en UCIP, de la necesidad de analgesia e inicio de tolerancia precoz

Objectives: Craniofacial clefts surgery associates a painful postoperative pain whose management is complicated with conventional analgesia. Material and methods: A parent controlled analgesia system was implanted with a continuous perfusion of tramadol, ondansetron and metamizole adjusted by weight. Parents are allowed to administer additional boluses if they observe irritability. We compared the variables of the cleft patients operated before and after the implantation of the system in our center. Results: During 2016, 16 craniofacial clefts were operated (4 cheilorhinoplasties and 12 palatal clefts). No PCA (parent controlled analgesia) system was used. The average time of stay in PICU was 1.5 days. It took an average of 2.5 days to initiate tolerance. The mean of VAS (Visual Analogic Scale) was 3. 53% required major opioids (morphine, fentanyl) not being sufficient analgesia every 3 hours. During 2017, 7 palatal fissures and 4 cheilorhinoplasties were operated (11). Both of them were controlled by PCA. Patients with palatal cleft were admitted to the PICU with a total mean of 0.5 days. The beginning of tolerance was advanced to the first postoperative day. The VAS diminished to 0.5. Only one patient required opioids. 72% did not need to associate any type of analgesia. Conclusions: The PCA system is a safe and risk-free insurance for analgesia of fissured patients with benefits such as: decrease in pain, stay in PICU, the need for analgesia and initiation of early tolerance

Combined immunotherapy encompassing intratumoral poly-ICLC, dendritic-cell vaccination and radiotherapy in advanced cancer patients.

Background: Combination immunotherapy has the potential to achieve additive or synergistic effects. Combined local injections of dsRNA analogues (mimicking viral RNA) and repeated vaccinations with tumor-lysate loaded dendritic cells shows efficacy against colon cancer mouse models. In the context of immunotherapy, radiotherapy can exert beneficial abscopal effects. Patients and methods: In this two-cohort pilot phase I study, 15 advanced cancer patients received two 4-week cycles of four intradermal daily doses of monocyte-derived dendritic cells preloaded with autologous tumor lysate and matured for 24 h with poly-ICLC (Hiltonol), TNF-α and IFN-α. On days +8 and +10 of each cycle, patients received intratumoral image-guided 0.25 mg injections of the dsRNA-analogue Hiltonol. Cyclophosphamide 600 mg/m2 was administered 1 week before. Six patients received stereotactic ablative radiotherapy (SABR) on selected tumor lesions, including those injected with Hiltonol. Expression of 25 immune-relevant genes was sequentially monitored by RT-PCR on circulating peripheral blood mononuclear cell (PBMCs) and serum concentrations of a cytokine panel were sequentially determined before and during treatment. Pre- and post-treatment PBMC from patients achieving durable stable disease (SD) were studied by IFNγ ELISPOT-assays responding to tumor-lysate loaded DC and by TCRß sequencing. Results: Combined treatment was, safe and well tolerated. One heavily pretreated castration-resistant prostate cancer patient experienced a remarkable mixed abscopal response to SABR+ immunotherapy. No objective responses were observed, while nine patients presented SD (five of them in the six-patient radiotherapy cohort). Intratumoral Hiltonol increased IFN-ß and IFN-α mRNA in circulating PBMC. DC vaccination increased serum IL-12 and IL-1ß concentrations, especially in patients presenting SD. IFNγ-ELISPOT reactivity to tumor lysates was observed in two patients experiencing durable SD. Conclusions: This radio-immunotherapy combination strategy, aimed at resembling viral infection in tumor tissue in combination with a dendritic-cell vaccine and SABR, is safe and shows immune-associated activity and signs of preliminary clinical efficacy.

Changes in serum interleukin-8 (IL-8) levels reflect and predict response to anti-PD-1 treatment in melanoma and non-small-cell lung cancer patients.

BACKGROUND: Surrogate biomarkers of efficacy are needed for anti-PD1/PD-L1 therapy, given the existence of delayed responses and pseudo-progressions. We evaluated changes in serum IL-8 levels as a biomarker of response to anti-PD-1 blockade in melanoma and non-small-cell lung cancer (NSCLC) patients. PATIENTS AND METHODS: Metastatic melanoma and NSCLC patients treated with nivolumab or pembrolizumab alone or nivolumab plus ipilimumab were studied. Serum was collected at baseline; at 2-4 weeks after the first dose; and at the time-points of response evaluation. Serum IL-8 levels were determined by sandwich ELISA. Changes in serum IL-8 levels were compared with the Wilcoxon test and their strength of association with response was assessed with the Mann-Whitney test. Accuracy of changes in IL-8 levels to predict response was estimated using receiver operation characteristics curves. RESULTS: Twenty-nine melanoma patients treated with nivolumab or pembrolizumab were studied. In responding patients, serum IL-8 levels significantly decreased between baseline and best response (P <0.001), and significantly increased upon progression (P =  0.004). In non-responders, IL-8 levels significantly increased between baseline and progression (P =  0.013). Early changes in serum IL-8 levels (2-4 weeks after treatment initiation) were strongly associated with response (P <0.001). These observations were validated in 19 NSCLC patients treated with nivolumab or pembrolizumab (P =  0.001), and in 15 melanoma patients treated with nivolumab plus ipilimumab (P <0.001). Early decreases in serum IL-8 levels were associated with longer overall survival in melanoma (P =  0.001) and NSCLC (P =  0.015) patients. Serum IL-8 levels also correctly reflected true response in three cancer patients presenting pseudoprogression. CONCLUSIONS: Changes in serum IL-8 levels could be used to monitor and predict clinical benefit from immune checkpoint blockade in melanoma and NSCLC patients.

Total and mutated EGFR quantification in cell-free DNA from non-small cell lung cancer patients detects tumor heterogeneity and presents prognostic value.

Mutation analysis of epidermal growth factor receptor (EGFR) gene is essential for treatment selection in non-small cell lung cancer (NSCLC). Analysis is usually performed in tumor samples. We evaluated the clinical utility of EGFR analysis in plasma cell-free DNA (cfDNA) from patients under treatment with EGFR inhibitors. We selected 36 patients with NSCLC and EGFR-activating mutations. Blood samples were collected at baseline and during treatment with EGFR inhibitors. Wild-type EGFR, L858R, delE746-A750, and T790M mutations were quantified in cfDNA by droplet digital PCR. Stage IV patients had higher total circulating EGFR copy levels than stage I (3523 vs. 1003 copies/mL; p < 0.01). There was high agreement for activating mutations between baseline cfDNA and tumor samples, especially for L858R mutation (kappa index = 0.679; p = 0.001). In 34 % of advanced NSCLC patients, we detected mutations in cfDNA not previously detected in tumor samples and double mutations in 17 %. Patients with baseline total EGFR copy levels above the median presented decreased overall survival (OS) (341 vs. 870 days, p < 0.05) and progression-free survival (PFS) (238 vs. 783 days; p < 0.05) compared with those with total EGFR copy levels below the median. Patients with baseline concentrations of activating mutations above the median (94 copies/mL) had lower OS (317 vs. 805 days; p < 0.05) and PFS (195 vs. 724 days; p < 0.05). During follow-up, T790M resistance mutation was detected in 53 % of patients. Total and mutated EGFR analysis in cfDNA seems a relevant tool to characterize the molecular profile and prognosis of NSCLC patients harboring EGFR mutations.

Evaluación de la percepción de riesgo en el personal de enfermeraí en la atención de pacientes sospechosos de riesgo biológico IV / Evaluation of the perception of risk in the nursing staff in care of patients with suspected biological hazard

Introducción. El personal sanitario es una población especialmente vulnerable a los riesgos derivados del ejercicio de su profesión. La complejidad que tiene la atención de los pacientes con virus Ébola lleva al estudio de la percepción de riesgo de este colectivo. Objetivo. Conocer la percepción de riesgo en el personal de enfermería que atendió a los pacientes con sospecha de Fiebre Hemorrágica Virus Ébola (FHVE). Material y métodos. Estudio transversal realizado en el Hospital Clínic de Barcelona. Se incluyeron 19 profesionales de enfermería que atendieron directamente a pacientes con sospecha de FHVE. Los datos se recogieron mediante un cuestionario de evaluación dimensional del riesgo percibido junto a variables sociodemográficas y un posterior análisis estadístico. Resultados. Un 68 % del personal de enfermería refirió tener un nivel de conocimiento elevado del riesgo asociado al factor de estudio. Un 42 % determinó que había una posibilidad muy alta de experimentar un daño. Un 63 % apreció que las consecuencias más nocivas se experimentarían a corto plazo y, finalmente, un 48 % valoró que había un riesgo muy alto de accidente o enfermedad asociado al factor de estudio, frente a un 5 % que lo valoró muy bajo. Conclusiones. Las respuestas obtenidas ponían de manifiesto que los profesionales de enfermería poseían un nivel de conocimiento muy alto sobre el riesgo asociado al factor de estudio, y que el temor emocional era también muy elevado (AU)

Introduction. Healthcare personnel is especially vulnerable to the risks derived from their job. The complexity that has the care of patients with Ebola justifies the study of the perception of risk of such professionals. Objective. To know the perception of risk in the nursing staff that takes care of patients suspected of suffering the Ebola virus. Material and methods. Transversal study carried out in the Hospital Clínic of Barcelona. 19 professionals of nursing staff that attended suspected Ebola patients were included in the study. The data was collected through a questionnaire of dimensional evaluation of the perceived risk along with sociodemographic variables and a subsequent statistics analysis. Results. A percentage of 68 % of the nursing staff refereed having a high level of knowledge of the risk associated with the factor of this study. A percentage of 42 % determined that there was a very high possibility of being harmed. A percentage of 63 % considered that the most harmful consequences would appear in short term, and finally a percentage of 48 % considered that there was a very high risk of accident or illness associated to the factor of this study, whereas a percentage of 5 % considered the risk to be very low. Conclusions. The answers obtained showed not only that nursing staff had a very high level of knowledge regarding the risk associated to the factor of this study, but also that the emotional fear was very high (AU)

Estudio del daño renal en el paciente pediátrico con vejiga neurógena y su relación con el patrón de funcionamiento vesical y el tratamiento recibido / Study of kidney damage in paediatric patients with neurogenic bladder and its relationship with the pattern of bladder function and treatment received

Objetivos: El fallo renal es la principal causa de morbimortalidad en pacientes con mielodisplasia. Analizamos la presencia de lesiones renales en la gammagrafía (DMSA) de estos pacientes y la relacionamos con el tipo de funcionamiento vesical, así como con el retraso en recibir un manejo adecuado. Material y métodos: Realizamos un estudio retrospectivo de pacientes con mielodisplasia en nuestro hospital desde 2004. Analizamos datos epidemiológicos, clínicos y el patrón de funcionamiento vesical según estudios urodinámicos. Clasificamos a los pacientes en 4 patrones urodinámicos según el comportamiento del detrusor y del esfínter; y lo relacionamos con la función renal en la gammagrafía y el manejo recibido desde el nacimiento. Resultados: Estudiamos 39 pacientes con mielodisplasia. El patrón vesical más frecuente fue el tipo A (61,5%) con hiperactividad del esfínter y del detrusor, seguido del D (20,5%), C (7,8%) y B (5,1%). El 38% de nuestros pacientes (n = 15) presenta algún tipo de nefropatía. El 92,9% de los niños que reciben tratamiento adecuado en el primer año de vida, no presentan lesiones renales en la gammagrafía. Encontramos algún tipo de nefropatía en el 56% de los pacientes en los que el tratamiento adecuado se demora más de un año; siendo la nefropatía más severa cuanto más tarde se inicia el manejo. Conclusiones: Existe una relación estadísticamente significativa entre el retraso en el tratamiento y la alteración en la gammagrafía renal en pacientes con vejiga neurógena. Es fundamental el estudio y tratamiento precoz de los pacientes para disminuir el deterioro renal, disminuir la necesidad de cirugía y mejorar las opciones de continencia

Objectives: Kidney failure is the main cause of morbidity and mortality in patients with myelodysplasia. We analysed the presence of renal lesions in these patients using dimercaptosuccinic acid scintigraphy and related their presence with the type of vesical function and the delay in receiving appropriate management. Material and methods: We performed a retrospective study of patients with myelodysplasia treated in our hospital since 2004. We analysed the epidemiological and clinical data and the pattern of bladder function according to urodynamic studies. We classified the patients into 4 urodynamic patterns according to detrusor and sphincter behaviour. We linked this behaviour to renal function in the scintigraphy and the care received since birth. Results: The study included 39 patients with myelodysplasia. The most common bladder pattern was type A (61.5%), with sphincter and detrusor hyperactivity, followed by type D (20.5%), C (7.8%) and B (5.1%). Some 38% of our patients (n = 15) had some type of nephropathy. Some 92.9% of the children who were properly treated during the first year of their life had no renal lesions in the scintigraphy. We found some type of nephropathy in 56% of the patients for whom appropriate treatment was delayed for more than a year. The nephropathy was more severe the later the management was started. Conclusions: There is a statistically significant relationship between a delay in treatment and impairment in renal scintigraphy in patients with neurogenic bladders. The early study and treatment of patients is essential for decreasing renal impairment, reducing the need for surgery and improving the continence options

Combinación de propranolol oral y láser de colorante pulsado en el tratamiento de los hemangiomas infantiles ulcerados / Combination of pulsed dye laser and propranolol in the treatment of ulcerated infantile haemangioma

INTRODUCCIÓN: La ulceración se trata de la complicación más frecuente de los hemangiomas infantiles, produciéndose en el 15,8% de los mismos, habitualmente en el apogeo de la fase proliferativa. Existen múltiples herramientas terapéuticas para el manejo de los hemangiomas ulcerados, sin que se haya demostrado una mayor eficacia de ninguna de ellas. Presentamos nuestra experiencia en el tratamiento con láser de colorante pulsado asociado a propranolol en los hemangiomas infantiles ulcerados. MATERIAL Y MÉTODOS: Realizamos un estudio observacional retrospectivo de pacientes con hemangioma infantil ulcerado que recibieron tratamiento con láser de colorante pulsado asociado a propranolol. Estudiamos 7 pacientes; 3 con hemangiomas en la zona labial y 4 en la zona del pañal. Asimismo, revisamos una cohorte de 5 niños con hemangiomas ulcerados de similares características que solo recibieron tratamiento con propranolol, curas tópicas y vendaje oclusivo. RESULTADOS: El tamaño medio de las úlceras fue de 1cm, con un tiempo medio de evolución previo al tratamiento de 2 semanas. En todos los casos había dolor y sangrado. Tras 2 semanas de tratamiento combinado con propranolol y láser, todas las úlceras se habían curado. El dolor desaparece tras la primera sesión de láser. Los pacientes con hemangioma ulcerado en la zona labial presentaron mejor respuesta que aquellos que lo presentaban en la zona del pañal. La cohorte de pacientes tratados únicamente con propranolol, curas tópicas y vendaje oclusivo precisaron un tiempo medio para la curación de 5,2 semanas. CONCLUSIONES: Nuestros resultados evidencian que el tratamiento combinado de láser de colorante pulsado y propranolol presenta un efecto sinérgico, acelerando la curación de los hemangiomas ulcerados. Se necesitarían más estudios, con un mayor número de pacientes, para confirmar estos datos

INTRODUCTION: Ulceration is the most common complication of infantile haemangioma, with 15.8% of them usually appearing in the proliferative phase. They can be managed in several ways. We present our experience in the treatment of ulcerated haemangioma with the combination of pulsed dye laser and propranolol. MATERIAL AND METHODS: A retrospective observational study was conducted on patients with ulcerated infantile haemangioma treated with pulsed dye laser in association with propranolol. The study included 7 patients, 3 cases in labial area and 4 cases in the nappy area. A review was also performed on a historical cohort of 5 children with ulcerated haemangiomas with the same features, but treated only with propranolol, topical agents and occlusive dressings. RESULTS: The median size of the ulcer was 1.0cm, and there was a mean time of onset pre-treatment of 2 weeks. Pain and bleeding was present in all patients. After 2 weeks of combined propranolol and laser treatment, all lesions were healed. The pain disappeared after the first laser session. Patients with ulcerative haemangioma in the labial area obtained a better response than patients with haemangioma in the nappy area. The cohort of patients treated with propranolol required a mean healing time of 5.2 weeks, with the addition of an occlusive dressing with ointment. CONCLUSIONS: We believe that our results suggest that combined treatment, laser and propranolol, has synergistic effects that accelerate the healing of ulcerated haemangioma, as observed in our patients. Further studies with larger numbers of patients are needed to confirm this fact

[Combination of pulsed dye laser and propranolol in the treatment of ulcerated infantile haemangioma]. / Combinación de propranolol oral y láser de colorante pulsado en el tratamiento de los hemangiomas infantiles ulcerados.

INTRODUCTION: Ulceration is the most common complication of infantile haemangioma, with 15.8% of them usually appearing in the proliferative phase. They can be managed in several ways. We present our experience in the treatment of ulcerated haemangioma with the combination of pulsed dye laser and propranolol. MATERIAL AND METHODS: A retrospective observational study was conducted on patients with ulcerated infantile haemangioma treated with pulsed dye laser in association with propranolol. The study included 7 patients, 3 cases in labial area and 4 cases in the nappy area. A review was also performed on a historical cohort of 5 children with ulcerated haemangiomas with the same features, but treated only with propranolol, topical agents and occlusive dressings. RESULTS: The median size of the ulcer was 1.0 cm, and there was a mean time of onset pre-treatment of 2 weeks. Pain and bleeding was present in all patients. After 2 weeks of combined propranolol and laser treatment, all lesions were healed. The pain disappeared after the first laser session. Patients with ulcerative haemangioma in the labial area obtained a better response than patients with haemangioma in the nappy area. The cohort of patients treated with propranolol required a mean healing time of 5.2 weeks, with the addition of an occlusive dressing with ointment. CONCLUSIONS: We believe that our results suggest that combined treatment, laser and propranolol, has synergistic effects that accelerate the healing of ulcerated haemangioma, as observed in our patients. Further studies with larger numbers of patients are needed to confirm this fact.

Study of kidney damage in paediatric patients with neurogenic bladder and its relationship with the pattern of bladder function and treatment received.

OBJECTIVES: Kidney failure is the main cause of morbidity and mortality in patients with myelodysplasia. We analysed the presence of renal lesions in these patients using dimercaptosuccinic acid scintigraphy and related their presence with the type of vesical function and the delay in receiving appropriate management. MATERIAL AND METHODS: We performed a retrospective study of patients with myelodysplasia treated in our hospital since 2004. We analysed the epidemiological and clinical data and the pattern of bladder function according to urodynamic studies. We classified the patients into 4 urodynamic patterns according to detrusor and sphincter behaviour. We linked this behaviour to renal function in the scintigraphy and the care received since birth. RESULTS: The study included 39 patients with myelodysplasia. The most common bladder pattern was type A (61.5%), with sphincter and detrusor hyperactivity, followed by type D (20.5%), C (7.8%) and B (5.1%). Some 38% of our patients (n=15) had some type of nephropathy. Some 92.9% of the children who were properly treated during the first year of their life had no renal lesions in the scintigraphy. We found some type of nephropathy in 56% of the patients for whom appropriate treatment was delayed for more than a year. The nephropathy was more severe the later the management was started. CONCLUSIONS: There is a statistically significant relationship between a delay in treatment and impairment in renal scintigraphy in patients with neurogenic bladders. The early study and treatment of patients is essential for decreasing renal impairment, reducing the need for surgery and improving the continence options.

Severe haematological complications during treatment with natalizumab.

The safety profile of natalizumab has been widely discussed due to several cases of progressive multifocal leukoencephalopathy, reported worldwide. Since the launch of natalizumab, 32 patients have been treated at our centre. In this context, we describe two cases (6.25%), one of immune-mediated acute haemolytic anaemia (IAHA) and another of immune thrombocytopenic purpura during treatment with natalizumab. The temporal relationship between drug administration and the nature of the haematological complications, confirmed with the serological findings in the case of the IAHA, suggests that natalizumab is the most probable cause for these adverse events. Although very uncommon, the haematological complications are severe enough to justify a close and careful monitoring for all patients with multiple sclerosis treated with an immunosuppressant treatment.