Retraso Diagnóstico en Oncología Pediátrica

Fueron revisados retrospectivamente las historias clínicas de 550 pacientes menores de 19 años de edad con diagnóstico citohistológico de malignidad, admitidos entre 01/01/1980 a 31/12/2000. La revisión de datos incluyó: tiempo del inicio de síntomas a la primera consulta (retraso familiar), tiempo desde primera consulta médica hasta la referencia a Centro especializado (con o sin diagnóstico de enfermedad tumoral) (retraso médico) y el tiempo desde la admisión al Centro de Referencia hasta el diagnóstico (Retraso del Centro de Referencia). Del total de 550 pacientes 204 (36%) reclutados en el Grupo A (ingresados entre 1979-89) y 346 (64%) en Grupo B (ingresados entre 1990-2000). De ellos, 189 (92.5%) del grupo A y 342 (98.5%) del grupo B (n: 531) fueron evaluables. Diecinueve pacientes (3.4%) fueron excluidos debido a datos incompletos (8 pacientes), sin seguimiento o referido a otro centro luego del diagnóstico (11 pacientes). En esta serie fue claro que aquellos pacientes con diagnóstico temprano (menor de 30 días) tuvieron una sobrevida alta (83.5%), siendo menor con retrasos mayores (53.3% en 30-60 días y 30.8% en retrasos mayores del 90 días), pero el periodo de seguimiento del Grupo A (1979- 1989) fue más largo. Igualmente se notifica que el incremento de la sobrevida entre ambos períodos fue comparado (1979- 89 vs. 1990-2000) fue de 54.8% al 72.8%, sin cambios significativos en el retraso diagnóstico. Esta observación sostiene el hecho de que los avances terapéuticos pueden disminuir el efecto de los retrasos. De cualquier manera, el retraso diagnóstico determina cambios metabólicos y nutricionales, y progresion de la enfermedad que pueden prevenirse con el uso protocolos cortos y agresivos. La pobre respuesta a la to quimioterapia es más frecuente en pacientes con estadios avanzados of disease. Dichos pacientes también presentan regularmente complicaciones infecciosas y malnutricion que resulta en pobre tolerancia al tratamiento.We retrospectively reviewed the charts of 550 patients of less than 19 years of age with cyto-histological diagnosis of malignancy, admitted from January 1979 to December2000. Retrieved data included: time from the beginning of symptoms to first consultation (family delay), time from first medical consultation to reference to the specialized Centre (with or without diagnosis of tumor disease) (Medical delay) and time from admission at the Reference Centre to diagnosis (Reference Centre delay). Of the grand total of 550 patients 204 (36%) belonged to group A (1979-89) and 346 (64%) to group B (1990-2000). Of these, 189 (92.5%) of group A and 342 (98.5%) of group B (n:531) were adequate for evaluation. Nineteen patients (3.4%) were excluded due to incomplete data (8 patients), no follow-up, or referral to another center after diagnosis (11 patients). As grand total from this series it is clear that those patients with early diagnosis (less than 30 days) had a high survival (83.5%), while this number decreased with longer delays (53.3% in 30-60 days and 30.8% in longer than 90 days), but the period of follow-up of Group A (1979-1989) was longer. At the same it was noticed that the survival increased when both periods were compared (1979-89 vs. 1990-2000) from 54.8% to 72.8%, even with no significant changes in the diagnostic delay. This observation sustains the fact that therapeutic advances may disregard the delays. Anyway, delay in diagnosis determines metabolic and nutritional changes, and progression of the disease all of which prevent the use of aggressive and short therapeutic protocols. So, poor response to chemotherapy is more frequent in patients with advanced stages of disease. These patients also present more regularly infectious complications and malnutrition which result in poor tolerance to the treatment

Histiocitosis de células de Langerhans en el raquis infantil

Introducción: la Histiocitosis de células de Langerhans incluye un amplio espectro de enfermedades de etiología desconocida, observada preponderantemente en niños. El objetivo del presente trabajo es: analizar formas de presentación, estudios complementarios, procedimientos diagnósticos, criterios de estadificación y resultados de distintas modalidades terapéuticas en 12 pacientes con Histiocitosis de células de Langerhans con localización raquídea. Materiales y Métodos: doce pacientes evaluados retrospectivamente con diseño de caso-control, tratados entre 1983- 2004. Seguimiento promedio 5 años. La estadificación incluyó extensión anatómica (Weinstein-Boriani-Biagini) y comportamiento biológico (Enneking). El centellograma, la TC y la RNM fueron los estudios de elección. El tratamiento incluyó corticoides, radioterapia, quimioterapia y cirugía. Resultados: relación masculino-femenino 10-2, media edad 9 años 5 meses. Datos clínicos relevantes: dolor, limitación funcional o deformidad y compromiso neurológico. Predominio de formas únicas en raquis lumbar, cuerpo vertebral. Las variables clínicas fueron modificadas favorablemente con significancia estadística (p < 0,05). Se obtuvo la remisión de la enfermedad sin excepciones, pero persistieron distintas secuelas morfológicas vertebrales, sin traducción clínica. Conclusión: Se obtuvo la curación de las lesiones, independientemente del tratamiento realizado. Al momento del diagnóstico, ningún criterio clínico, histológico o de imágenes pudo predecir el curso ulterior de la enfermedad.Background: Langerhans' Cell Histiocytosis includes a wide spectrum of disorders of unknown etiology observed mostly in children. The purpose of this investigation is: to analyze the clinical course, the complementary studies, the diagnostic procedures, the criteria of stadification and the results of the diverse treatment modalities, in the spine involvement. Methods: twelve pediatric patients treated from 1983 to 2004 were retrospectively reviewed through a case-control design. Mean follow up was 5 years. Lesions were classified according to their anatomical extent (Weinstein-Boriani-Biagini) and biological behavior (Enneking). Supports for staging and searching were taken from Scintigraphy, CT and MRI. Treatment was performed by surgery, chemotherapy, corticosteroids and radiation therapy, depending on the extent of the disease. Alpha was set at 0.05. Results: male-female ratio was 10-2. The most frequent clinical data were pain, deformity or stiffness and neurological impairment. Solitary location was prevalent. The lumbar spine was most frequently affected. The vertebral body was involved in all the cases. Clinical data were improved without exceptions but vertebral body collapse underwent poor improvement. Conclusions: recurrence free-healing was obtained in all cases, regardless of treatment method. At the time of diagnosis, no clinical, radiographic or histological criteria could predict the course of the disease

Design considerations for CRBRP heat transport system piping operating at elevated temperatures

The heat transport system sodium piping for the Clinch River Breeder Reactor Plant (CRBRP) within the reactor containment building must withstand high temperatures for long periods of time. Each phase of the mechanical design process of the piping system is influenced by elevated temperature considerations which include material thermal creep effects, ratchetting caused by rapid temperature transients and stress relaxation, and material degradation effects. The structural design philosophy taken to design the CRBRP piping operating in a high temperature environment is described. The resulting design of the heat transport system piping is presented along with a discussion of special features that resulted from the elevated temperature considerations

Tumors of the Oralfaucial Cavity in Children. A review of 156 patients

Se presenta un estudio retrospectivo de 156 pacientes (pac) con tumores que se manifestaron en la cavidad oral, con localización inicial en maxilares o partes blandas (se excluyeron los tumores de la cara sin compromiso bucal). La edad promedio fue 9 años y 6 meses (rango de 1 día a 17 años). El 62% se presentó en varones. La localización inicial de los tumores fue ósea y en partes blandas en el 50% de los casos respectivamente; 102 pacientes tuvieron lesiones benignas y 54 lesiones malignas. Los manifestaciones al ingreso fueron: tumor palpable o visible (43%), tumor + dolor (22%), dolor (13%) y otros como caída de dientes, parálisis, fiebre o asimetría facial (22%). La rutina de estudio comprendió radiografía panorámica de maxilar, centellografía ósea (gamma-cámara) con Tecnesio 99, tomografía axial computada (TAC) y resonancia nuclear magnética (RNM). Los pacientes fueron tratados en forma multidisciplinaria siendo la cirugía (punción aspiración con aguja fina, biopsia y/o resección) el procedimiento inicial en la mayoría de ellos. El manejo se realizó basándose en un algoritmo como muestra la (figura 1).We reviewed the findings of 156 patients (pat) with tumors involving the oral cavity representing lesions of bones and soft tissues (cases of tumors involving the face but without involvement of the oral cavity were excluded). Mean age was 9 years and 6 months (1d-17y); 62% presented in males. Bones (50%) and soft tissues (50%) were almost equally represented as the initial site. One hundred two patients presented benign tumors and 54 with malignancies. Symptoms and signs on admission were: palpable or obvious mass (43%), tumorassociated pain (22%), pain (13%), and other (spontaneous missing of teeth, paralysis, fever and facial asymmetry) (22%). Imagining studies included X-ray of the maxilla, bone scintiscan, CAT scan, and MRI. Patients were assisted by a multidisciplinary team being surgery (FNAB, biopsy, resection) the first procedure for most of the patients. This was followed by an algorhythm - designed approach (figure 1).Facultad de OdontologíaFacultad de Ciencias Médica

Complicación inusual de un homoinjerto en posición pulmonar

The implantation of a homograft in pulmonary position is commonly used to resolve the obstruction of the outfl ow tract of the right ventricle in various forms of complex congenital heart diseases. Unfortunately often they have dysfunction mainly due to stenosis, leading to indicate its replacement with a consequent increase in morbidity and mortality of repeated surgeries. Angioplasty with stent homograft appears as a therapeutic option to prolong the useful life and to postpone surgery. We report the case of a young woman who required multiple interventions for correction of pulmonary atresia with ventricular septal defect.El implante de un homoinjerto en posición pulmonar se utiliza habitualmente para resolver la obstrucción del tracto de salida del ventrículo derecho en varias formas de cardiopatías congénitas complejas. Lamentablemente suelen presentar disfunción principalmente por estenosis, lo que conlleva a indicar su reemplazo con el consiguiente aumento de la morbimortalidad de repetidas cirugías. La angioplastia con stent del homoinjerto aparece entonces como una opción terapéutica para prolongar su vida útil y posponer la cirugía. Se presenta el caso de una mujer de 21 años que requirió múltiples intervenciones para la corrección de una atresia pulmonar con comunicación interventricular

Cáncer en el adolescente: a propósito de nuestra experiencia (224 pacientes)

El cáncer es una enfermedad de rara ocurrencia en pediatría y la adolescencia (10-19 años). Casi el 70% de las neoplasias ocurren en pacientes menores de 5 años. Presentamos nuestra experiencia sobre 224 pacientes adolescentes con una enfermedad neoplásica no hematológica. El 61% correspondieron al sexo masculino. La incidencia relativa de las neoplasias fue: tumores del sistema nervioso central 30,5%, sarcomas de partes blandas 22,7%, tumores de hueso 18,1%, tumores germinales 11,8%, carcinomas 6,3%, nefroblastoma 2,9% y neuroblastoma 0,9%. Comparando dos períodos diferentes (1984-89 y 1990-99) la sobrevida libre de enfermedad (SLE) a 48 meses fue de 39,4% (45/114) y 81,8% (90/110), respectivamente mientras que 49% y 35,5% presentaron secuelas, respectivamente. La adaptación social posterior a la curación orgánica de la enfermedad mostró que sólo 4/135 pacientes (2,9%) están en la Universidad; el grupo más numeroso correspondió a aquellos con instrucción primaria incompleta (42,2% versus 33%). El 37% y 53,3% respectivamente, trabajan (intermitentemente) y el 13,3% y 15,5% han formado pareja. De estos últimos, 20 pacientes, el 35% tiene 10 hijos; 6 son mujeres y 3 tenían tumores pelvianos, 2 de ellas de ovario (tumores germinales).Cancer is of rare occurrence in pediatrics and adolescence (10- 19 years of age). Within this group almost 70% of the neoplasms present in patients less than 5 years of age. We are reporting our experience on 224 adolescents with one solid tumor. 61% were males. The relative incidence of the lesions was: tumors of the central nervous system 30.5%, soft tissue sarcomas 22.7%, bone tumors 18.1%, germ-cell tumors 11.8%, carcinomas 6.3%, nephroblastoma 2.9%, and neuroblastoma 0.9%. Comparing two time periods (1984-89 and 1990-99) the free of disease survival time at 48 months was 39.4% (45/114) and 81.8% (90/110), respectively; 49% and 35.5% presented sequelae, respectively. Social adaptation after cure of the disease revealed that only 4/135 patients (2.9%) are at the University, while 37% and 53.3% are working (intermittently) and 13.3% and 15.5% live as a couple, respectively. In the latter group, 20 patients, 35% have 10 children; 6 are women, 3 had had pelvic tumors 2 of the in the ovary (germ-cell tumors)

Dyslipidemia and changes in lipid profiles associated with rheumatoid arthritis and initiation of anti–tumor necrosis factor therapy

Objective To investigate the frequency of lipid testing in clinical practice and to explore the relationship between rheumatoid arthritis (RA), dyslipidemia, and other cardiovascular (CV) risk factors with RA treatment. Methods Patients in this retrospective database study were ages ≥18 years and had ≥2 physician diagnoses for RA or osteoarthritis (OA; comparator group) between March 2004 and March 2008. Outcomes of interest included the percentage of RA and OA patients receiving lipid tests, lipid profiles (total cholesterol, low‐density lipoprotein [LDL] cholesterol, and high‐density lipoprotein [HDL] cholesterol) of RA versus OA patients, and lipid profiles of RA patients before and after initiation with a tumor necrosis factor (TNF) inhibitor. We used multivariable regression to control potential confounders between the cohorts. Results Over a median ≥2‐year followup, fewer RA patients than OA patients had ≥1 lipid test (62.0% [95% confidence interval (95% CI) 61.5–62.5] versus 69.8% [95% CI 69.5–70.1]). Mean total cholesterol and LDL cholesterol were each 4 mg/dl lower in the RA cohort ( P < 0.0001); HDL cholesterol was similar between the cohorts. Across the RA cohort, 25.2% of patients had suboptimal LDL cholesterol levels (≥130 mg/dl). Among RA patients not receiving lipid‐lowering therapy who initiated TNF inhibitor therapy (n = 96), mean total cholesterol and LDL cholesterol increased by 5.4 and 4.0 mg/dl, respectively. Conclusion Patients with RA were less likely to be tested for hyperlipidemia and had more favorable lipid profiles than patients with OA. TNF inhibitor therapy modestly increased all lipid parameters. Additional studies are needed to determine the effect of traditional CV risk factors and inflammation and the impact of biologic agents on CV outcomes in RA patients.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/93521/1/21693_ftp.pd

Prenatally Detected Congenital Perineal Mass Using 3D Ultrasound which was Diagnosed as Lipoblastoma Combined with Anorectal Malformation: Case Report

We report a case of prenatally diagnosed congenital perineal mass which was combined with anorectal malformation. The mass was successfully treated with posterior sagittal anorectoplasty postnatally. On ultrasound examination at a gestational age of 23 weeks the fetal perineal mass were found on the right side. Any other defects were not visible on ultrasonography during whole gestation. Amniocentesis was performed to evaluate the fetal karyotyping and acetylcholinesterase which were also normal. As the fetus grew up, the mass size was slowly increased more and more. At birth, a female neonate had a perineal mass on the right side as expected. During operation, the anal sphincteric displacement was found near the mass and reconstructed through posterior sagittal incision. This is the first reported case of prenatally diagnosed congenital perineal mass, after birth which was diagnosed as lipoblastoma and even combined with anorectal malformation. This case shows that it can be of clinical importance to be aware of this rare fetal perineal mass in prenatal diagnosis and counseling

Impact of COVID-19 on cardiovascular testing in the United States versus the rest of the world

Objectives: This study sought to quantify and compare the decline in volumes of cardiovascular procedures between the United States and non-US institutions during the early phase of the coronavirus disease-2019 (COVID-19) pandemic. Background: The COVID-19 pandemic has disrupted the care of many non-COVID-19 illnesses. Reductions in diagnostic cardiovascular testing around the world have led to concerns over the implications of reduced testing for cardiovascular disease (CVD) morbidity and mortality. Methods: Data were submitted to the INCAPS-COVID (International Atomic Energy Agency Non-Invasive Cardiology Protocols Study of COVID-19), a multinational registry comprising 909 institutions in 108 countries (including 155 facilities in 40 U.S. states), assessing the impact of the COVID-19 pandemic on volumes of diagnostic cardiovascular procedures. Data were obtained for April 2020 and compared with volumes of baseline procedures from March 2019. We compared laboratory characteristics, practices, and procedure volumes between U.S. and non-U.S. facilities and between U.S. geographic regions and identified factors associated with volume reduction in the United States. Results: Reductions in the volumes of procedures in the United States were similar to those in non-U.S. facilities (68% vs. 63%, respectively; p = 0.237), although U.S. facilities reported greater reductions in invasive coronary angiography (69% vs. 53%, respectively; p < 0.001). Significantly more U.S. facilities reported increased use of telehealth and patient screening measures than non-U.S. facilities, such as temperature checks, symptom screenings, and COVID-19 testing. Reductions in volumes of procedures differed between U.S. regions, with larger declines observed in the Northeast (76%) and Midwest (74%) than in the South (62%) and West (44%). Prevalence of COVID-19, staff redeployments, outpatient centers, and urban centers were associated with greater reductions in volume in U.S. facilities in a multivariable analysis. Conclusions: We observed marked reductions in U.S. cardiovascular testing in the early phase of the pandemic and significant variability between U.S. regions. The association between reductions of volumes and COVID-19 prevalence in the United States highlighted the need for proactive efforts to maintain access to cardiovascular testing in areas most affected by outbreaks of COVID-19 infection