The Alagille syndrome is one of the most common inherited disorders causing chronic liver disease during childhood. During the 1990s, 38 children with Alagille syndrome were evaluated at two pediatric centers in Buenos Aires, Argentina. Characteristic clinical, humoral, and cutaneous features were analyzed. The average age of diagnosis was 29 months old (range of between 2 months and 15 years). Cholestasis was evident in 92% of patients during the neonatal period. Family antecedents related to the syndrome were found in 18.5% of the patients. Peculiar facies developed in 85% of patients. Chronic cholestasis and pruritus were observed in all of the patients and jaundice was evident in 78%. Eighty-four percent of the patients had heart disease (pulmonary stenosis, intraauricular communication, intraventricular communication), 76% of them showed growth retardation, and vertebrae abnormalities were found in 63%. Embryotoxon appeared in 76% of patients, and renal disturbances in 21%. Eleven children (28%) had xanthomas, in the neck, elbows, palms, helixes, inguinal area, gluteus, and knees. The earliest findings appeared in the first months of life, and the latest at 5 years of age. The xanthomas located in the folds had a stony aspect. Cholesterol levels ranging from 220 to 1600 mg percentage (mg%) were demonstrated in all of the children with xanthomas. Liver transplantation was performed in seven of the patients (18.4%). Two of them died after this operation. The disappearance of xanthomas after transplantation was remarkable in all of the patients.

Summary:Secondary myelodysplastic syndromes (MDS) are increasingly being reported after autologous transplantation. Transient dysplastic changes have also been observed after this type of treatment. However, to the best of our knowledge no systematic morphological analysis has been perfomed to determine the influence of stem cell transplantation on bone marrow morphology. In 53 patients undergoing autologous transplantation, we evaluated the bone marrow, before and 6 and 12 months after the transplant, in order to analyze the appearance of dyshemopoietic changes, assessed according to a pre-established score. We also studied 25 bone marrow samples obtained at the time of diagnosis, prior to treatment, but we did not find morphological atypia. Six months after transplant, cellularity and thrombopoiesis had decreased in 38% and 49% of patients respectively, although 1 year after the process they were normal in most cases. Myelodysplasia was already present in bone marrow before transplantation and continued to be in evidence for a long time afterwards. This suggests that chemotherapy and radiotherapy used prior to transplantation are responsible for dysplastic changes. The myeloid line was the most affected with 100% of patients showing dysgranulopoiesis 1 year after autografting. Cytopenias were observed in 51% and 44% of patients 6 and 12 months after transplantation. Moreover, concomitant presence of cytopenia and myelodysplasia was observed in 37.7% of patients at 6 months after transplantation and 25% at 12 months, and therefore they could be diagnosed with MDS. These data contrast with the incidence of secondary MDS reported in earlier publications. According to these findings, the value of the FrenchAmerican-British Co-operative Group criteria for the diagnosis of MDS following autologous transplantation is questionable. Moreover, since dyshemopoietic features are almost always present after autologous transplant, morphological criteria are not useful for early

BackgroundPain is a frequent symptom with many types of cancer. In 80–85% of patients with metastatic prostate cancer (MPC), the cancer spreads to the bone, which causes pain, pathologic fractures or spinal cord compression.PurposeTo develop a pharmaceutical care programme and to analyse the prevalence of pain in patients with MPC who attended the outpatient area of the hospital pharmacy.Material and methodsA literature search in several databases was conducted (Pubmed, Medline and Google Scholar) for a review of pain in patients with MPC. We also consulted the websites of the National Cancer Institute and other cancer organisations. The Wisconsin Brief Pain Questionnaire was selected for characterising the types and degree of pain that patients experienced. A diary to be distributed to patients to record their pain in terms of degree, duration and analgesic treatment, and an educational pamphlet about oncologic pain that included advice on how to deal with the pain and better understand its symptomatology, were developed. Follow-up of patients with MPC who began receiving treatment with abiraterone or enzalutamide from the hospital pharmacy from January to July 2016 was performed. They completed a questionnaire designed to evaluate the type of pain they were experiencing at their initial visit and at subsequent visits. They were given the diary and an informational pamphlet.ResultsThe programme began with 38 patients, of whom 56% presented with bone metastases. From the questionnaires they completed at each visit, it was observed that 35% reported pain. The most common analgesic treatment used was NSAIDs (36%) or NSAIDs plus opioids (29%). The materials patients received improved characterisation of their pain and served as a psychological support, basically through understanding that pain was a frequent symptom of their illness and one that could be treated more effectively.ConclusionA high prevalence of pain in patients with MPC was identified which indicates that analgesic treatment is often inadequate. Creating pharmaceutical care programmes may contribute to better pain evaluation and treatment, and better support for patients.References and/or acknowledgementsThe author would like to thank the pharmacists who supported this study.No conflict of interest

INTRODUCCIÓNHasta el año 1996 la evolución de los paciente infectados por el virus de la inmunodeficiencia humana (VIH) era hacia estadio de sida y la mayor parte acababan falleciendo en un corto espacio de tiempo. La introducción de los tratamientos antirretrovirales de gran actividad (TARGA) ha disminuido la morbimortalidad y aumentado la supervivencia, y con ello el desarrollo de enfermedades que previamente no se observaban dado su tiempo de latencia. En este grupo están las enfermedades hepáticas y sus complicaciones, producto de la infección crónica de virus hepatotropos (VHB y VHC generalmente), cuya infección esta muy ligada a la del VIH (1).En esta revisión vamos a dedicarnos exclusivamente al virus de la hepatitis C (VHC), ya que se ha convertido en una de las principales causas de ingreso hospitalario y muerte en el grupo de pacientes con infección por el VIH (2,3). BIOLOGÍATanto el VIH como el VHC son RNA virus y presentan una cinética viral muy similar. Ambos tienen una gran variabilidad genética y alta tasa de mutación, debido a que carecen de enzimas correctores de errores de replicación. La diferencia principal entre ellos es que el VIH integra el genoma en la [0212-7199 (2005) RESUMENEl tratamiento de la infección por el virus de la hepatitis C (VHC) en los pacientes coinfectados por el virus de la inmunodeficiencia humana (VIH) se ha convertido en un tema de gran importancia, ya que las complicaciones de la hepatopatía son actualmente la primera causa de mortalidad entre el colectivo con infección por el VIH.El objetivo de este trabajo es revisar los datos biológicos y epidemiológicos de la coinfección, y sobre todo las pautas de tratamiento a seguir, valorando las complicaciones producidas por los fármacos utilizados y sus interacciones, e informar de los resultados de los principales estudios realizados.La pauta de tratamiento actualmente aceptada incluye interferón pegilado y ribavirina. Con estos fármacos han mejorado los resultados respecto a pautas previas, aunque la respuesta es variable según el genotipo -VHC que presente el paciente.PALABRAS CLAVE: VHC. VIH. Coinfección HCV-VIH. Interferón. Ribavirina. ABSTRACT Treatment of chronic hepatitis C Virus (HCV) infection in human immunodeficiency virus (HIV) infected