Enfermedad mínima residual en leucemia mieloide aguda no promielocítica: Resultados preliminares de un estudio colaborativo internacional / Minimal residual disease in non-myelocytic acute myeloid leukemia: preliminary results of an international collaborative study

A pesar de los avances en los protocolos de tratamiento y en las medidas de soporte en pacientes con Leucemia Mieloide Aguda (LMA), 27% presentan recaídas de la enfermedad. Esto se debe, entre otras causas, a la persistencia de pequeñas cantidades de células malignas (blastos) resistentes a la terapia. Estas pequeñas cantidades de blastos remanentes se denominan Enfermedad Mínima Residual (EMR). La determinación de EMR requiere de técnicas no solo muy sensibles, sino también específicas, y permite evaluar la respuesta individual a la terapia. La introducción de la EMR como parámetro de respuesta y estratificación está bien definida en Leucemia Linfoblástica Aguda (LLA). Por el contrario, aunque existen publicaciones sobre el impacto pronóstico de la EMR en LMA, aún no se encuentra incluida en forma sistemática en los protocolos nacionales actuales, entre otros motivos, por lo laborioso de la determinación y por la necesidad de validación de la misma. Debe tenerse en cuenta que el inmunofenotipo de los blastos mieloides suele ser más heterogéneo que el de los blastos en LLA, presentando, en muchos casos, subpoblaciones diferentes entre sí, lo cual dificulta su detección certera y no hay consenso definido en cuanto a la metodología más eficaz. En este trabajo describimos una nueva estrategia de marcación y análisis estandarizada en un estudio multicéntrico internacional para LMA y la utilidad de la EMR como parámetro de respuesta y de estratificación. Asimismo, detallamos los resultados preliminares de nuestra cohorte de pacientes (AU)

Despite the improvement in treatment and supportive care of patients with Acute Myeloid Leukemia (AML), 27% of them relapse. This is due to the persistence of small amounts of malignant cells (blasts) resistant to therapy, among other causes. These small amounts of blasts are called Minimal Residual Disease (MRD). The determination of MRD requires not only techniques with high sensitivity but also with high specificity, and allows to evaluate the individual response to treatment. The introduction of MRD as a response parameter is well established in Acute Lymphoblastic Leukemia (ALL), and it is used in current stratification protocols. On the other hand, even though there are some reports regarding the prognostic impact of MRD in AML, it is still not included in the current national protocols due to the lack of validation of the determination, among other causes. This is due to the fact that the immunophenotype of myeloid blasts is more heterogeneous than in ALL, presenting different subpopulations, which difficults their accurate detection. Thus, there is still no consensus regarding the most effective approach. In this article, we describe a new staining and analysis strategy standardized by an international multicentric study, and the utility of EMR as a response and stratification parameter. Additionally, we show the preliminary results of our patient cohort. (AU)

Hallazgos incidentales en medicina genómica / Incidental Findings in Genomic Medicine

Las técnicas de Biología Molecular de última generación, como es la secuenciación masiva en paralelo o NGS (Next Generation Sequencing), permite obtener gran cantidad de información genómica, la cual muchas veces va más allá de la detección de una variante patogénica en un gen que explique la patología (hallazgo primario). Es así como surgió desde hace años la discusión internacional respecto a la decisión a tomar frente a los hallazgos secundarios accionables, es decir, aquellos hallazgos de variantes clasificadas como patogénicas o probablemente patogénicas que no están relacionadas con el fenotipo del paciente, pero que tiene alguna medida preventiva o tratamiento posible y, por lo tanto, podría ser de utilidad para la salud del paciente. Luego de revisar la bibliografía internacional y debatir entre los expertos del Hospital de Pediatría Garrahan, se logró establecer una política institucional y reforzar el hecho de que se trata de una disciplina multidisciplinaria. Así, fue posible definir que solo se atenderá las cuestiones relacionadas con la edad pediátrica, dejando para un tratamiento posterior aquellas variantes detectadas en genes que sean accionables en edad adulta. En el Hospital Garrahan, ha sido posible definir claramente cómo proceder frente a los hallazgos secundarios, al adaptar el consentimiento informado a esta necesidad, definiendo cuándo serán informados, y sabiendo que serán buscados intencionalmente en los genes clínicamente accionables enlistados en la última publicación del American College of Medical Genetics and Genomics, siempre y cuando el paciente/padre/tutor lo consienta (AU)

The latest generation of molecular biology techniques, including massive parallel sequencing or NGS (Next Generation Sequencing), allows us to obtain a whealth of genomic information, which often goes beyond the detection of a pathogenic variant in a gene that explains the pathology (primary finding). As a result, an international discussion has arisen over the years regarding the decision-making concerning actionable secondary findings, it means, those findings of variants classified as pathogenic or probably pathogenic that are not related to the patient's phenotype, but which have some possible preventive measure or treatment and, therefore, could be useful for the patient's health. After reviewing the international literature and discussing among the experts of the Hospital de Pediatría Garrahan, an institutional policy was established and the concept that this is a multidisciplinary discipline was reinforced. Consequently, it has been defined that only issues related to children will be addressed, reserving those variants detected in genes that are actionable in adulthood for later treatment. At Garrahan Hospital, we were able to clearly define how to proceed with secondary findings by adapting the informed consent to this need, defining when they will be reported, and knowing that they will be intentionally searched for in the clinically actionable genes listed in the latest publication of the American College of Medical Genetics and Genomics, as long as the patient/parent/guardian consents (AU)

[Efficacy, effectiveness and safety of the adjuvanted influenza vaccine in the population aged 65 or over]. / Eficacia, efectividad y seguridad de la vacuna antigripal adyuvada en población de 65 o más años de edad.

Most of the complications and deaths related to seasonal flu occur in the elderly population (≥65 years) with comorbidities, and the influenza vaccine is the most effective way to prevent them. Immunization is less effective in older adults due to immunosenescence. MF59-adjuvanted vaccines, designed to improve the magnitude, persistence and amplitude of the immune response in elderly people, have been used in clinical practice since 1997 in their trivalent formulation and, since 2020, in their tetravalent formulation. Data from various studies show that these vaccines are not only safe for all age groups, with a reactogenicity profile similar to that of the conventional vaccine, but also that they are especially effective in boosting the immune response in the population aged 65 or over by increasing antibody titers after vaccination and significantly reducing the risk of hospital admission. Adjuvanted vaccines have been shown to provide cross-protection against heterologous strains and to be as effective as the high-dose vaccine in the population aged 65 or over. In this review, the scientific evidence on the efficacy and effectiveness of the MF59-adjuvanted vaccine in real clinical practice in people ≥65 years of age is analyzed through a narrative and descriptive review of the literature with data from clinical trials, observational studies and systematic reviews or meta-analysis.

First Reported Case of Malignant Ectomesenchymoma with p.Leu122Arg Mutation in MYOD1 Gene: Extensive Intra- and Extracranial Tumor in a 15-Year-Old Female.

BACKGROUND: Ectomesenchymomas (EMs) are extremely rare neoplasms composed of malignant mesenchymal components and neuroectodermal derivatives. They are described in a wide variety of locations, with the head and neck region being one of the most frequently involved areas. EMs are usually managed as high-risk rhabdomyosarcomas and have similar outcomes. METHODS: We present the case of a 15-year-old female with an EM that arose in the parapharyngeal space and extended into the intracranial space. RESULTS: Histologically, the tumor presented an embryonal rhabdomyosarcomatous mesenchymal component and the neuroectodermal component was constituted by isolated ganglion cells. Next-generation sequencing (NGS) revealed a p.Leu122Arg (c.365 T > G) mutation in the MYOD1 gene, a p.Ala34Gly mutation in the CDKN2A gene, and CDK4 gene amplification. The patient was treated with chemotherapy. She died 17 months after the debut of symptoms. CONCLUSION(S): To our knowledge, this is the first reported case in English literature of an EM with this MYOD1 mutation. We suggest combining PI3K/ATK pathway inhibitors in these cases. NGS should be performed in EMs cases to detect mutations with potential treatment options.

Gallbladder disease and pancreatic cancer risk: a multicentric case-control European study.

BACKGROUND AND AIMS: The overall evidence on the association between gallbladder conditions (GBC: gallstones and cholecystectomy) and pancreatic cancer (PC) is inconsistent. To our knowledge, no previous investigations considered the role of tumour characteristics on this association. Thus, we aimed to assess the association between self-reported GBC and PC risk, by focussing on timing to PC diagnosis and tumour features (stage, location, and resection). METHODS: Data derived from a European case-control study conducted between 2009 and 2014 including 1431 PC cases and 1090 controls. We used unconditional logistic regression models to estimate odds ratios (ORs) and corresponding 95% confidence intervals (CIs) adjusted for recognized confounders. RESULTS: Overall, 298 (20.8%) cases and 127 (11.6%) controls reported to have had GBC, corresponding to an OR of 1.70 (95% CI 1.33-2.16). The ORs were 4.84 (95% CI 2.96-7.89) for GBC diagnosed <3 years before PC and 1.06 (95% CI 0.79-1.41) for ≥3 years. The risk was slightly higher for stage I/II (OR = 1.71, 95% CI 1.15-2.55) vs. stage III/IV tumours (OR = 1.23, 95% CI 0.87-1.76); for tumours sited in the head of the pancreas (OR = 1.59, 95% CI 1.13-2.24) vs. tumours located at the body/tail (OR = 1.02, 95% CI 0.62-1.68); and for tumours surgically resected (OR = 1.69, 95% CI 1.14-2.51) vs. non-resected tumours (OR = 1.25, 95% CI 0.88-1.78). The corresponding ORs for GBC diagnosed ≥3 years prior PC were close to unity. CONCLUSION: Our study supports the association between GBC and PC. Given the time-risk pattern observed, however, this relationship may be non-causal and, partly or largely, due to diagnostic attention and/or reverse causation.

GEIS-SEHOP clinical practice guidelines for the treatment of rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma (STS) in children and adolescents. In Spain the annual incidence is 4.4 cases per million children < 14 years. It is an uncommon neoplasm in adults, but 40% of RMS are diagnosed in patients over 20 years of age, representing 1% of all STS in this age group. RMS can appear anywhere in the body, with some sites more frequently affected including head and neck, genitourinary system and limbs. Assessment of a patient with suspicion of RMS includes imaging studies (MRI, CT, PET-CT) and biopsy. All patients with RMS should receive chemotherapy, either at diagnosis in advanced or metastatic stages, or after initial resection in early local stages. Local control includes surgery and/or radiotherapy depending on site, stage, histology and response to chemotherapy. This guide provides recommendations for diagnosis, staging and treatment of this neoplasm.

Genetic predisposition to fetal and neonatal cancer

Background Neonatal tumors represent an extremely rare and heterogeneous disease with an unknown etiology. Due to its early onset, it has been proposed that genetic factors could play a critical role; however, germline genetic analysis is not usually performed in neonatal cancer patients Patients and methods To improve the identification of cancer genetic predisposition syndromes, we retrospectively review clinical characteristics in 45 patients with confirmed tumor diagnosis before 28 days of age, and we carried out germline genetic analysis in 20 patients using next-generation sequencing and directed sequencing. Results The genetic studies did not find any germline mutation except patients diagnosed with bilateral retinoblastoma who harbored RB1 germline mutations. Conclusions Our results suggest that genetic factors have almost no higher impact in most neonatal tumors. However, since the heterogeneity of the tumors and the small sample size analyzed, we recommend complementary and centralized germline studies to discard the early onset as an additional criterion to take into account to improve the identification of cancer genetic predisposition syndromes in neonates (AU)

Genetic predisposition to fetal and neonatal cancer.

BACKGROUND: Neonatal tumors represent an extremely rare and heterogeneous disease with an unknown etiology. Due to its early onset, it has been proposed that genetic factors could play a critical role; however, germline genetic analysis is not usually performed in neonatal cancer patients PATIENTS AND METHODS: To improve the identification of cancer genetic predisposition syndromes, we retrospectively review clinical characteristics in 45 patients with confirmed tumor diagnosis before 28 days of age, and we carried out germline genetic analysis in 20 patients using next-generation sequencing and directed sequencing. RESULTS: The genetic studies did not find any germline mutation except patients diagnosed with bilateral retinoblastoma who harbored RB1 germline mutations. CONCLUSIONS: Our results suggest that genetic factors have almost no higher impact in most neonatal tumors. However, since the heterogeneity of the tumors and the small sample size analyzed, we recommend complementary and centralized germline studies to discard the early onset as an additional criterion to take into account to improve the identification of cancer genetic predisposition syndromes in neonates.

Factors of the epidemiological triad that influence the persistence of human papilloma virus infection in women with systemic lupus erythematosus.

We studied the epidemiologic triad-related factors influencing human papilloma virus (HPV) persistence in Mexican women with systemic lupus erythematosus (SLE). Patients aged ≥18 years with SLE (American College of Rheumatology criteria), with and without HPV persistence, were selected. Groups were analyzed by (1) host: clinical disease characteristics; (2) agent: (I) infectious (prevalence, incidence, HPV genotype and co-infections (≥2 HPV genotypes or mycoplasmas)), (II) chemical (contraceptives and immunosuppressive drugs) and (III) physical (vitamin D deficiency) and (3) environment. A total of 121 SLE patients were selected over a two-year period. (1) Host: mean age 45.8 years and disease duration 12.7 years. (2) Agent: (I) infectious. HPV infection prevalence in the second sample was 26.4%, high-risk HPV genotypes 21.5% and co-infections 7.4%. HPV infection incidence was 13.2%, persistence 13.2% and clearance 15.7%. (II) Chemical: use of oral hormonal contraceptives 5% and immunosuppressive treatment 97.5%. (III) Physical: Vitamin D levels were similar in both groups. (3) Environment: (I) natural. A total of 60.6% of patients were residents of Puebla City. (II) Social: The mean education level was 10.9. Poverty levels were: III degree 52.4%, IV degree 28% and II degree 17%. (III) Cultural behavioral: Onset of sexual life was 20.5 years, 10% had ≥3 sexual partners and 51.2% were postmenopausal. In conclusion, no factor of the epidemiologic triad was associated with HPV infection prevalence.

Agranulocitosis en paciente en tratamiento con oxcarbacepina que, tras su retirada, se evidenció recuperación de neutrófilos / Agranulocytosis in a patient in oxcarbazepine treatment, after its withdrawal, neutrophils returned to a normal levels

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Risk of pancreatic cancer associated with family history of cancer and other medical conditions by accounting for smoking among relatives.

Background: Family history (FH) of pancreatic cancer (PC) has been associated with an increased risk of PC, but little is known regarding the role of inherited/environmental factors or that of FH of other comorbidities in PC risk. We aimed to address these issues using multiple methodological approaches. Methods: Case-control study including 1431 PC cases and 1090 controls and a reconstructed-cohort study (N = 16 747) made up of their first-degree relatives (FDR). Logistic regression was used to evaluate PC risk associated with FH of cancer, diabetes, allergies, asthma, cystic fibrosis and chronic pancreatitis by relative type and number of affected relatives, by smoking status and other potential effect modifiers, and by tumour stage and location. Familial aggregation of cancer was assessed within the cohort using Cox proportional hazard regression. Results: FH of PC was associated with an increased PC risk [odds ratio (OR) = 2.68; 95% confidence interval (CI): 2.27-4.06] when compared with cancer-free FH, the risk being greater when ≥ 2 FDRs suffered PC (OR = 3.88; 95% CI: 2.96-9.73) and among current smokers (OR = 3.16; 95% CI: 2.56-5.78, interaction FHPC*smoking P-value = 0.04). PC cumulative risk by age 75 was 2.2% among FDRs of cases and 0.7% in those of controls [hazard ratio (HR) = 2.42; 95% CI: 2.16-2.71]. PC risk was significantly associated with FH of cancer (OR = 1.30; 95% CI: 1.13-1.54) and diabetes (OR = 1.24; 95% CI: 1.01-1.52), but not with FH of other diseases. Conclusions: The concordant findings using both approaches strengthen the notion that FH of cancer, PC or diabetes confers a higher PC risk. Smoking notably increases PC risk associated with FH of PC. Further evaluation of these associations should be undertaken to guide PC prevention strategies.

Bloqueo del plano transverso del abdomen ecoguiado: una técnica anestésico-analgésica en cirugía abdominal / Ultrasound-guided transversus abdominis plane block in abdominal surgery

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Micosis fungoide. Experiencia en un hospital pediátrico / Mycosis Fungoides: Experience in a Pediatric Hospital

La micosis fungoide (MF) es el linfoma cutáneo primario de células T más frecuente. Su aparición en la infancia es excepcional. Objetivos: Describir las características epidemiológicas, clínicas, histopatológicas e inmunofenotípicas de los pacientes con MF. Describir los tratamientos utilizados y la evolución. Material y método: Se incluyeron todos los pacientes admitidos en el Hospital de Pediatría Dr. J. P. Garrahan (Argentina) en el período comprendido entre agosto de 1988 y julio de 2014 con diagnóstico clínico e histopatológico de MF. Resultados: Se diagnosticaron 14 pacientes con MF. La distribución por sexo fue M/F: 1:1,33. La edad media al diagnóstico fue de 11,23 años (rango: 8 a 15 años). El tiempo promedio de evolución hasta el momento del diagnóstico fue de 3 años y 6 meses (rango: 4 meses a 7 años). Todos los pacientes presentaron la forma clínica hipopigmentada y en el 42% se asoció la forma clásica. El 50% (n = 7) exhibió un inmunofenotipo CD8 positivo de forma exclusiva. El 78% presentó estadio IB. La fototerapia fue el tratamiento de elección. Cuatro pacientes tuvieron por lo menos una recaída y 3 demostraron progresión de su enfermedad a nivel cutáneo. La evolución fue favorable en todos los casos. Conclusiones: La MF es una entidad infrecuente en la infancia, siendo la forma hipopigmentada la más frecuente. Su diagnóstico es tardío debido a la similitud con otras enfermedades hipopigmentadas frecuentes en la niñez. A pesar de tener un buen pronóstico, presenta alta tasa de recidivas y requiere un seguimiento a largo plazo (AU)

Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is unusual in children. Objectives: We aimed to describe the epidemiologic, clinical, histopathologic, and immunophenotypic characteristics of MF as well as treatments and course of disease in a pediatric case series. Material and method: Data for all patients admitted to our pediatric hospital (Hospital Dr. J. P. Garrahan) in Argentina with a clinical and histopathologic diagnosis of MF between August 1988 and July 2014 were included. Results: A total of 14 patients were diagnosed with MF. The ratio of boys to girls was 1:1.33. The mean age at diagnosis was 11.23 years (range, 8-15 years). The mean time between onset and diagnosis was 3.5 years (range, 4 months-7 years). All patients had hypopigmented MF and 42% also presented the features of classic MF. Seven (50%) had the CD8+ immunophenotype exclusively. Seventy-eight percent were in stage IB at presentation. Phototherapy was the treatment of choice. Four patients relapsed at least once and skin lesions progressed in 3 patients. All patients improved. Conclusions: MF is unusual in children. The hypopigmented form is the most common. Diagnosis is delayed because the condition is similar to other hypopigmented diseases seen more often in childhood. Although prognosis is good, the rate of recurrence is high, so long-term follow-up is necessary (AU)

Why does measles persist in Europe?

Several reasons may explain why measles was not eliminated by 2015 and continues to persist in Europe, including low measles vaccination coverage, low anti-measles herd immunity levels, and the mobility of individuals with measles across Europe. The study assessed the mean measles vaccination coverage in the European Union and the WHO European region with one and two doses of measles vaccine from 1980 to 2015, and the prevalence of vaccine-induced protected individuals and anti-measles herd immunity levels in the WHO European region during 2000-2015. The study found that measles vaccination coverage with two doses of vaccine was <90% in the European Union and the WHO European region from 1995 to 2015. In the WHO European region, the two-dose measles vaccination coverage during the 2000-2015 period was <95% in 41 countries (79%), and the prevalence of vaccine-induced protected individuals in the target vaccination population was lower than the herd immunity threshold of 94.4% in 33 countries (63%). The additional vaccination coverage required to establish herd immunity in these countries ranged from 0.2% to 18%. Two of the factors explaining measles persistence in Europe in 2015 were the two-dose measles vaccination coverage <95% and the prevalence of individuals with vaccine-induced protection of <94.4% in most countries of the WHO European region during the 2000-2015 period. Screening and vaccination programs should be developed to detect and immunize susceptible individuals in areas and population groups without anti-measles herd immunity in all European countries.

A systems approach identifies time-dependent associations of multimorbidities with pancreatic cancer risk.

BACKGROUND: Pancreatic ductal adenocarcinoma (PDAC) is usually diagnosed in late adulthood; therefore, many patients suffer or have suffered from other diseases. Identifying disease patterns associated with PDAC risk may enable a better characterization of high-risk patients. METHODS: Multimorbidity patterns (MPs) were assessed from 17 self-reported conditions using hierarchical clustering, principal component, and factor analyses in 1705 PDAC cases and 1084 controls from a European population. Their association with PDAC was evaluated using adjusted logistic regression models. Time since diagnosis of morbidities to PDAC diagnosis/recruitment was stratified into recent (<3 years) and long term (≥3 years). The MPs and PDAC genetic networks were explored with DisGeNET bioinformatics-tool which focuses on gene-diseases associations available in curated databases. RESULTS: Three MPs were observed: gastric (heartburn, acid regurgitation, Helicobacter pylori infection, and ulcer), metabolic syndrome (obesity, type-2 diabetes, hypercholesterolemia, and hypertension), and atopic (nasal allergies, skin allergies, and asthma). Strong associations with PDAC were observed for ≥2 recently diagnosed gastric conditions [odds ratio (OR), 6.13; 95% confidence interval CI 3.01-12.5)] and for ≥3 recently diagnosed metabolic syndrome conditions (OR, 1.61; 95% CI 1.11-2.35). Atopic conditions were negatively associated with PDAC (high adherence score OR for tertile III, 0.45; 95% CI, 0.36-0.55). Combining type-2 diabetes with gastric MP resulted in higher PDAC risk for recent (OR, 7.89; 95% CI 3.9-16.1) and long-term diagnosed conditions (OR, 1.86; 95% CI 1.29-2.67). A common genetic basis between MPs and PDAC was observed in the bioinformatics analysis. CONCLUSIONS: Specific multimorbidities aggregate and associate with PDAC in a time-dependent manner. A better characterization of a high-risk population for PDAC may help in the early diagnosis of this cancer. The common genetic basis between MP and PDAC points to a mechanistic link between these conditions.

Cancer sincronico de endometrio y trompa / Synchronous primary endometrial and tube fallopian cancers

Los tumores sincrónicos del tracto genital femenino son un entidad infrecuente que plantea un reto en el diagnóstico diferencial con la enfermedad metastásica. La mayoría de ellos son cánceres de endometrio y ovario, siendo los tumores sincrónicos de endometrio (CE) y trompa (CT) una asociación excepcional. Presentamos el caso de una paciente de 54 años con un diagnóstico preoperatorio de CE en la en la pieza quirúrgica se desveló la existencia de un tumor sincrónico de trompa izquierda. A propósito de este caso se realiza una revisión del tema haciendo hincapié en cómo llegar a un correcto diagnóstico de los tumores independientes descartando la extensión tumoral y la enfermedad metastásica.

Synchronous primary cancers of gynecological tract are uncommon and a challenge in the differential diagnosis with metastatic disease. Most of them are endometrial and ovarian cancers. Synchronous primary endometrial (EC) and tube fallopian cancers (TC) are a very rare association. We report the case of a patient of 54 years with EC preoperative diagnosis with synchronous left TC postoperative diagnosis. We review the topic emphasizing how to reach a correct diagnosis of tumors independent refusing the tumor invasion and metastatic disease.

Eficacia de las inyecciones intrarticulares de ácido hialurónico para el tratamiento de la artrosis de rodilla: resultados de una serie de casos tratados en una Unidad del Dolor / Effectiveness of intra-articular injections of hyaluronic acid for the treatment of knee osteoarthritis: results of a series of patients treated in a pain unit

Objetivo: Valorar los resultados del tratamiento de la artrosis de rodilla mediante una inyección intrarticular de ácido hialurónico en lo que se refiere a reducción del dolor, mejoría de la función articular y duración de los efectos. Método: Pacientes con diagnóstico clínico y radiológico de artrosis en diferentes fases de evolución en una o ambas rodillas, tratados en la Unidad del Dolor de un hospital de tercer nivel. Se recogió prospectivamente la información facilitada por el paciente siguiendo la práctica clínica habitual: características sociodemográficas y clínicas. Se realizaron valoraciones pre y postratamiento del dolor en reposo, a la deambulación y al subir/bajar escaleras, rigidez matutina, tiempo caminando y las horas/calidad del descanso nocturno. En la visita postratamiento se registraron, además, el grado de mejoría experimentada, duración de la misma y la satisfacción del investigador. El tratamiento consistió en una única inyección de ácido hialurónico por vía intrarticular, con posibilidad de una dosis de recuerdo. Resultados: Se incluyeron 29 pacientes (52 rodillas); en 23 se trataron ambas rodillas (n = 46) y en 6 una rodilla. El 92,3 % corresponden a articulaciones artrósicas con un tiempo de evolución > 12 meses, con grado clínico 4-5 en el 61,6 % de las rodillas tratadas (n = 32) y grado radiológico III-IV de Kellgren y Lawrence en el 67,7 % (n = 35), por lo que un 55,8 % de los casos fueron calificados como severos por los investigadores. Hubo una mejoría significativa en todos los parámetros de dolor y funcionalidad considerados (p < 0,001). El tiempo caminando se incrementó en más del 100 % y el 67,3 % de los pacientes definieron su calidad de sueño como normal, frente al 38,5 % inicial. La mejoría fue valorada por el investigador como buena o muy buena en el 73,1 % de los casos. Conclusiones: Nuestro trabajo confirma la eficacia de una única inyección de ácido hialurónico para reducir la sintomatología en pacientes con gonartrosis en diferentes grados de severidad, con resultados que se mantienen entre 6 y 12 meses y son especialmente significativos en aquellos con artrosis leve y moderada (AU)

Objective: To evaluate the results of treatment of knee osteoarthritis with a single intra-articular injection of hyaluronic acid in terms of pain reduction, joint function improvement and duration of effects. Method: Patients with clinical and radiological diagnosis of osteoarthritis at different stages of evolution in one or both knees were treated at the Pain Unit of a tertiary hospital. Sociodemographic and clinical characteristics provided by the patient following the usual clinical practice were prospectively collected. Pain at rest, walking, up and down stairs, morning stiffness, time walking as well as time/quality sleep were recorded pre and post-treatment. At post-treatment visit the degree and duration of improvement experienced, as well as the investigator assessment were also recorded. The treatment consisted of a single intra-articular injection of hyaluronic acid, with the possibility of a booster. Results: A total of 29 patients (52 knees) were included. Both knees were treated in 23 of them (n = 46) and only one knee in the remaining 6. The 92.3 % were osteoarthritic joints with an evolution > 12 months, with clinical grade 4-5 in 61.6 % (n = 32) and radiological grade III-IV of Kellgren & Lawrence in 67.7 % (n = 35), making 55.8 % of cases were classified as severe by the investigators. There was a significant improvement in all parameters of pain and function considered (p < 0.001). Walking time increased by over 100 % and 67.3 % of patients defined their quality of sleep as normal, compared to 38.5 % at baseline. The improvement was assessed by the investigator as good or very good in 73.1 % of cases. Conclusions: Our study confirms the effectiveness of a single injection of hyaluronic acid to reduce symptoms in patients with knee osteoarthritis with different grades of severity, with results that remain between 6 and 12 months and are especially significant in those patients with mild to moderate osteoarthritis (AU)

Mycosis Fungoides: Experience in a Pediatric Hospital. / Micosis fungoide. Experiencia en un hospital pediátrico.

Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is unusual in children. OBJECTIVES: We aimed to describe the epidemiologic, clinical, histopathologic, and immunophenotypic characteristics of MF as well as treatments and course of disease in a pediatric case series. MATERIAL AND METHOD: Data for all patients admitted to our pediatric hospital (Hospital Dr. J. P. Garrahan) in Argentina with a clinical and histopathologic diagnosis of MF between August 1988 and July 2014 were included. RESULTS: A total of 14 patients were diagnosed with MF. The ratio of boys to girls was 1:1.33. The mean age at diagnosis was 11.23 years (range, 8-15 years). The mean time between onset and diagnosis was 3.5 years (range, 4 months-7 years). All patients had hypopigmented MF and 42% also presented the features of classic MF. Seven (50%) had the CD8+ immunophenotype exclusively. Seventy-eight percent were in stage IB at presentation. Phototherapy was the treatment of choice. Four patients relapsed at least once and skin lesions progressed in 3 patients. All patients improved. CONCLUSIONS: MF is unusual in children. The hypopigmented form is the most common. Diagnosis is delayed because the condition is similar to other hypopigmented diseases seen more often in childhood. Although prognosis is good, the rate of recurrence is high, so long-term follow-up is necessary.

Fibromialgia y niveles de vitamina D / Fibromyalgia and vitamin D levels

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Lente intraocular fáquica plegable acrílica de apoyo angular para la corrección de miopía: seguimiento de 5 años / An angle-supported foldable phakic intraocular lens for correction of myopia: A five-year follow-up

OBJETIVO: Evaluar la eficacia y la seguridad de una lente intraocular (LIO) fáquica plegable de apoyo angular para la corrección de miopía de grado moderado-alto tras 5 años de seguimiento. MÉTODOS: Análisis prospectivo y retrospectivo, observacional, longitudinal, no aleatorizado de una serie casos que incluye un total de 100 ojos de 67 pacientes con miopía moderada-alta implantados con una LIO fáquica Acrysof Cachet (Alcon Laboratories Inc.) con el objetivo de minimizar el error refractivo. El rango de edad comprende de 18 a 60 años. La agudeza visual sin corrección de lejos (AVsc), la refracción manifiesta, la agudeza visual con corrección de lejos (AVcc), la densidad de células endoteliales, la posición de la LIO fáquica, la presión intraocular y las complicaciones detectadas fueron registradas antes de la operación y durante los 5 años de seguimiento. RESULTADOS: Cinco años después de la implantación la refracción manifiesta media se redujo de forma significativa de un equivalente esférico de −11,62 ± 3,35 dioptrías (D) a −0,33 ± 0,85 D. La AVsc fue de 20/20 o mejor en 5 de los 25 casos (20%) y 20/40 o mejor en 22 casos (88%). La AVcc fue de 20/20 o mejor en 17 casos (68%) y 20/32 o mejor en 23 casos (92% de los ojos). El error refractivo residual presentaba un valor entre ± 0,50 D y emetropía en 12 casos (48%) y entre ± 1,00 D en 19 casos (76%). La media de pérdida de células endoteliales en los 5 años fue del 11,8% en la región central y del 13,7% en la periferia. La distancia media entre endotelio-LIO fáquica fue de 2,11 ± 0,18 mm y la distancia media LIO fáquica-cristalino, de 0,88 ± 0,20 mm. CONCLUSIONES: Esta LIO fáquica de apoyo angular proporciona una corrección refractiva y una predictibilidad favorables, así como una seguridad aceptable en pacientes con un grado de miopía moderado-alto. A pesar de que la densidad de células endoteliales disminuyó durante los 5 años de seguimiento, los resultados están dentro del rango reportado en estudios anteriores con otras LIO fáquicas

OBJECTIVE: To evaluate the efficacy and safety of an angle-supported foldable phakic intraocular lens (pIOL) for the correction of moderate to high myopia after 5 years follow-up. METHODS: Prospective and retrospective, observational, longitudinal, non-randomised consecutive series of cases conducted on a total of 100 eyes of 67 patients with moderate to high myopia implanted with an Acrysof Cachet pIOL (Alcon Laboratories Inc.) with the aim of minimising the refractive error. The ages ranged between 18 to 60 years. Uncorrected distance visual acuity (UDVA), manifest refraction, corrected distance visual acuity (CDVA), endothelial cells density, pIOL position, intraocular pressure, and complications were recorded preoperatively and during the 5 year follow-up. RESULTS: Five years after implantation, the mean manifest spherical equivalent refraction reduced significantly from −11.62 ± 3.35 dioptres (D) to −0.33 ± 0.85 D. UDVA was 20/20 or better in 5 of 25 cases (20%), and 20/40 or better in 22 cases (88%). CDVA was 20/20 or better in 17 cases (68%), and 20/32 or better in 23 cases (92%) of eyes. The residual refractive error was within ± 0.50 D of emmetropia in 12 cases (48%), and within ± 1.00 D in 19 cases (76%). Mean endothelial cell loss at 5 years was 11.8% central, and 13.7% peripheral. Mean endotheliumpIOL distance was 2.11 ± 0.18 mm, and mean pIOL-crystalline distance was 0.88 ± 0.20 mm. CONCLUSIONS: This angle supported pIOL provided a favourable refractive correction and predictability, as well as acceptable safety in patients with moderate to high myopia. Although endothelial cell density decreased over 5 years, the results are within the range reported in previous studies with other pIOLs