Osteosarcoma multicéntrico sincrónico en paciente pediátrico: reporte de un caso / Synchronous multicenter osteosarcoma in a pediatric patient: Report of a case

El osteosarcoma es una neoplasia maligna común del tejido óseo. No obstante, la variante denominada multicéntrica o multifocal, es una entidad rara. Además, si al momento del diagnóstico se identifica más de una lesión se le añade el término sincrónico. Se presenta el primer caso en el Perú de una paciente pediátrica de 9 años referida a nuestro instituto con imágenes radiológicas sugestivas de neoplasia ósea en fémur distal derecho. Posteriormente, se diagnosticó osteosarcoma multicéntrico sincrónico mediante biopsia y exámenes imagenológicos. Recibió tratamiento quimioterapéutico triple con ifosfamida, doxorrubicina y cisplatino, sin mejora en la condición clínica. Posteriormente, desarrolló metástasis pulmonar y recibió tratamiento paliativo con ciclosofamida. En conclusión, el osteosarcoma multicéntrico sincrónico es un tipo de neoplasia ósea maligna, altamente agresiva y de mal pronóstico. Nuestra experiencia con esta triple terapia de quimioterapia no tuvo una buena respuesta clínica. Sugerimos una evaluación individual para el tratamiento de esta variante

Osteosarcoma is a common malignancy affecting bones. However, the multicenter or multifocal variety is a rare condition. Also, if at the time of diagnosis more than one lesion is identified, then the 'synchronous' term is added to the case definition. We present the first case described in Peru of a 9-year old patient who was referred to our institution with radiological images suggesting a bone malignancy in her distal right femur. Afterwards, a synchronous multicenter sarcoma was diagnosed with biopsy and image studies. She received triple chemotherapy with ifosfamide, doxorubicin, and cisplatin, but there was no improvement in her clinical status. Later she developed lung metastases and she received palliative therapy with cyclophosphamide. In conclusion, synchronous multicenter osteosarcoma is a highly aggressive bone malignancy with a poor prognosis. Our experience with triple therapy did not lead to a good clinical response. We suggest individual assessment for treating this variant

Recuperación nutricional post cirugía cardiaca en niños con cardiopatía congénita / Nutritional recovery after cardiac surgery in children with congenital heart disease

Resumen: Introducción: La desnutrición es frecuente en niños con cardiopatía congénita (CC). El manejo mé dico e intervenciones cardioquirúrgicas contribuyen a mejorar el estado nutricional en estos niños. Objetivo: Describir la recuperación nutricional posterior a cirugía de CC y sus factores asociados. Pacientes y Método: Estudio longitudinal. Se reclutaron 46 sujetos menores de 18 años ingresados para cirugía de CC entre abril de 2015 y abril de 2016. Se incluyeron las siguientes CC: comunica ción interventricular (CIV), comunicación interauricular (CIA), hipoplasia de ventrículo izquierdo (HVI), Tetralogía de Fallot (TOF) y transposición de grandes arterias (dTGA). Se excluyeron los síndromes genéticos y pacientes con otra enfermedad con compromiso nutricional. Se obtuvo al ingreso y los meses 1, 3 y 6 postquirúrgico los datos demográficos, de su CC, uso de sonda naso- gástrica (SNG), control nutricional, peso y talla. Los valores Z de indicadores antropométricos se calcularon según estándares de la OMS. Resultados: La mediana de edad de los pacientes reclutados fue de 8 meses (RIC 3,26); 24 (52%) varones; 6 (13%) prematuros y 12 (26,1%) pequeños para edad gestacional (PEG). Las CC fueron: 9 (19,6%) CIV; 8 (17,4%) CIA; 12 (26,1%) HVI; 9 (19,6%) TOF y 8 (17,4%) dTGA. ZP/T-IMC/E promedio -0,6 (± 1,5DE), 28,3% malnutrición por déficit. ZT/E promedio -0,86 (± 1,3 DE), 21,7% talla baja. Se encontraron diferencias según CC para edad, uso de SNG y tener control nutricional. Hubo una mejoría de ZT/E entre ingreso y tercer mes (p = 0,02) y de ZP/T-IMC/E al tercer (p = 0,046) y sexto mes (p = 0,001). Se disminuyó el uso de SNG, 19 al ingreso y 3 al sexto mes (p = 0,0016). Se encontró una correlación entre ZP/T-IMC/E de ingreso y una recuperación nutricional (r = -0,7; p < 0,001). Conclusiones: En este estudio se encontró una alta prevalencia de prematurez, PEG, malnutrición por déficit y talla baja, con recuperación de peso, pero no de talla post cardiocirugía.

Abstract: Introduction: Malnutrition is common in children with congenital heart disease (CHD). Medical treatment and surgical interventions contribute improving the nutritional status of these children. Objective: To describe nutritional recovery in children with CHD and associated factors after surgery. Patients and Method: Longitudinal study. 46 Children under 18 years old admitted for CHD surgery between April 2015 and April 2016 were recruited. The following CHD were included: Ventricular septal defect (VSD), Atrial septal defect (ASD), Hypoplastic left heart syndrome (HLHS), Tetralogy of Fallot (TOF), and Transposition of great arteries (dTGA). Children with genetic syndromes and other diseases that could compromise nutritional status were excluded. We obtained demographic, CHD, nasogastric tube use (NGT), nutritional evaluation, and weight and height data at the time of admission and one, three and six months after surgery and. Z-score to assess anthropometric measu res were calculated according to WHO standards. Results: Median age was 8 months (IQR: 3,26), 24 (52%) male, 6 (13%) preterm and 12 (26,1%) small for gestational age (SGA). CHD diagnosis were: 9 (19,6%) VSD, 8 (17,4%) ASD, 12 (26,1%) HLHS, 9 (19,6%) TOF and 8 (17,4%) dTGA. The mean weight-for-heigth-BMI-for-age-z-score (W/H-BMI/AZ) was 0,6 ± 1,5 SD, (28.3% of undernutri tion). The mean heigth-for-age-z-score (H/AZ) was -0,86 ± 1.3sd (21.7% of short stature). We found differences between each CHD and age, use of NGT and been under nutritional follow-up. There was an improvement between H/AZ at admission and 3rd month (p = 0,02), and W/H-BMI/AZ at 3th (p = 0,046) and 6th month (p = 0,001). Use of NGT decreased from admission to 6th month (19 vs 3) (p = 0,0016). We found correlation between admission W/H-BMI/AZ and nutritional recovery (r = -0,7; p < 0,001). Conclusion: There is a high prevalence of prematurity, SGA, undernutrition and short stature use of with weight recovery but not in heigth after cardio-surgery.

Diagnóstico nutricional en lactantes menores con cardiopatía congénita: comparación de dos clasificaciones antropométricas / Nutritional assessment in infants with congenital heart disease: comparison of two anthropometric classifications

INTRODUCCIÓN: Los niños con cardiopatía congénita (CC) presentan alto porcentaje de malnutrición por déficit, siendo difícil la interpretación de su estado nutricional. OBJETIVO: Describir el estado nutricional de lactantes con CC utilizando dos clasificaciones antropométricas y realizar una comparación entre ellas. PACIENTES Y MÉTODO: Estudio de cohorte no concurrente. Se estudiaron menores de 12 meses sometidos a cirugía de CC. Se excluyeron prematuros, pequeños para la edad gestacional, portadores de síndrome genético u otra enfermedad con compromiso nutricional. Se registraron datos demográficos, diagnóstico de CC, peso y talla de ingreso. Se realizó evaluación nutricional comparando estándares OMS según clasificación norma ministerial (CNM) y una Clasificación Antropométrica Integrada (CAI) que define desnutrición si (ZT/E)≤-2 y/o (ZP/T)≤-2, riesgo de desnutrición ZP/T entre -1 a -1,9, eutrofia ZP/T entre -0,9 a +0,9, sobrepeso entre +1 a +1,9 y obesidad ZP/T≥+2. RESULTADOS: Se incluyeron 387 intervenciones, 219 (56,6%) varones, mediana de edad 3,1 meses (RIC:0,4;6,4). Un 26,4% tenían talla baja. Utilizando CNM 55 sujetos presentaron dos diagnósticos por superposición de ZP/E y ZP/T, con CAI no hubo superposición. Al comparar CNM con CAI se encontró diferencia en desnutrición 28,9% versus 38,5%(p = 0,001), riesgo de des nutrición 27,4% versus 16,3%(p = 0,01) y obesidad 4,9% versus 3,3%(p = 0,03) respectivamente. Se encontró correlación entre ZP/E y ZP/T, r = 0,6(p < 0,001) y entre ZP/E y ZT/E, r = 0,6(p < 0,001). Conclusiones: Los niños con CC presentan alto porcentaje de desnutrición y talla baja. Utilizando las mismas mediciones antropométricas CAI no presentó superposición de diagnósticos y detectó más desnutrición. El P/E es útil como tamizaje, pero insuficiente en malnutrición crónica.

INTRODUCTION: Children with congenital heart disease (CHD) present a high percentage of undern utrition and the interpretation of their nutritional assessment is difficult. OBJECTIVE: To describe the nutritional status of infants with CHD using two anthropometric classifications and compare them. PATIENTS AND METHOD: Non-concurrent cohort study. We studied children under 12 months under going cardiac surgery. We excluded preterm infants, small for gestational age, carriers of genetic syndrome or other disease with nutritional compromise. Demographic data, type of CHD, weight and height were recorded. Nutritional assessment was performed using WHO standards per health ministry criteria (HMC) and per an Integrated Anthropometric Classification (IAC), which defines undernutrition if height-for-age Z-score (ZT/E)≤-2 and/or weight-for-height (ZP/T)≤-2, risk of un dernutrition as ZP/T between -1 to -1,9, normal as ZP/T between -0.9 to +0.9, overweight as ZP/T between +1 to +1.9 and obesity as ZP/T≥+2. RESULTS: 387 interventions were included, 219 (56.6%) were males, median age 3.1 months (IQR:0.4;6.4). A 26.4% presented short stature. Using HMC classification 55 subjects presented two diagnoses by overlap of ZP/E and ZP/T, although with IAC there was no overlap. Comparing HMC with IAC, a difference was found in undernutrition, 28.9% versus 38.5% (p = 0.001), risk of undernutrition 27.4% versus 16.3%(p = 0.01) and obesity 4.9% ver sus 3.3% (p = 0.03) respectively. Correlation was found between ZP/E and ZP/T, r = 0.6(p < 0.001) and between ZP/E and ZT/E, r = 0.6 (p < 0.001). CONCLUSIONS: Children with CHD have a high per centage of undernutrition and short stature. Using the same anthropometric measurements IAC did not present overlapping diagnoses and detected more undernutrition. P/E is useful as screening, but insufficient in chronic undernutrition.

Community Based Cross-Sectional Study Exploring The Beliefs Towards Voluntary Blood Donation In A Fully Literate District Of India.

Background: Voluntary non-remunerated blood donation is the key strategy to ensure availability of safe blood. India is facing shortage of 2.5 million units of blood annually and voluntary donation rate is only 50%. The present study was conducted in a fully literate district of India to assess the attitude and practice of blood donation and various associated beliefs. Methodology: This cross-sectional community based study was conducted in Cheranalloor Panchayat of Ernakulam. A total of 202 respondents selected using multi-stage sampling between 18 and 60 years of age were interviewed. Data was analyzed using computer. Results & Conclusion: 25.2% respondents ever donated blood which correlated with knowledge level (r=0.233) or attitude level (r=0.225). The major reasons for blood donation were for relatives (39.2%), in emergency for others (35.3%) and as voluntary donation (6.4%). Among non-willing, reasons cited for not willing to donate blood were ill health (46.9%), old age (25.8%) and fear of blood (16.6%). Attitude towards blood donation is low in this literate area. Proper information regarding blood donation needs to be conveyed to the community.

Adjuvant chemotherapy with six cycles of AC regimen versus three cycles of AC regimen followed by three cycles of Paclitaxel in node-positive breast cancer.

Context: Antracycline-Cyclophosphamide (AC) along with Paclitaxel/Docetaxel, either in combination or sequential regimens, is showing superior results than Anthracycline-containing regimens. Aims: This study was designed to determine whether adding Paclitaxel to a standard adjuvant chemotherapy regimen AC for breast cancer patients would prolong the time to recurrence and survival. Settings and Design: Randomized, prospective, open-labeled, single-institutional study. Materials and Methods: Fifty stage II breast cancer patients accruing 25 patients in each arm, treated between July 2007 and January 2010, were included in the study. Initial surgical treatment was Modified Radical Mastectomy. Systemic therapy was to have begun within 4-6 weeks of the patient's surgery. In the control arm, all the patients were treated with six cycles of adjuvant chemotherapy with AC regimen repeated at an interval of 3 weeks. For the study arm, the patients received adjuvant chemotherapy with three cycles of AC regimen followed by three cycles of Paclitaxel, repeated at an interval of 3 weeks. All the patients of both the arms received locoregional external beam radiotherapy (EBRT) after the entire course of chemotherapy. All the hormone receptor-positive patients received Tamoxifen. Statistical Analysis Used: Statistical analysis was performed using the chi-square test and the Kaplan Meier survival analysis with the log-rank (Mantel-Cox) test. Results: Adding Paclitaxel to AC resulted in a statistically significant disease-free survival. The overall survival was also improved significantly. The toxicity profile in both the arms was comparable. Conclusions: In early and node-positive breast cancer, the addition of three cycles of Paclitaxel after completion of three cycles of AC improves the disease-free and overall survival.

A case of disseminated hydatidosis.

A young lady initially found to have hydatid cysts in the lung only, subsequently within a very short period was found to develop cysts in the liver. Soon after, she developed cysts in the subcutaneous tissue over the anterolateral chest wall, which, on investigation, revealed hepatic cysts herniating through the chest wall defects caused by previous operations. The unique features of this case include the degree of dissemination, the multiplicity of sites and the peculiar nature of herniation of the hepatic cysts into the parietes.

The Repair of Critical-Size Defect with Fibrin Sealant in Rat Calvaria

This study was designed to evaluate the capacity of fibrin sealant to mediate the repair of critical size defects of rat calvaria. Twenty-four rats were randomized into four groups(n=6) and an 8-mm circular calvarial defect was made in each rat. In Group I and II, as control groups, defects were left untreated and evaluated at postoperative 6th and 12th week, respectively. In Group III and IV, defects were treated with fibrin sealant implantation and evaluated at the same point of time as in Group I and II, respectively. Results were examined by gross and histologic findings, simple radiographic study, and radiodensitometric analysis for bony density quantitatively. By gross and simple radiographic findings, animals with fibrin sealant implantation generally showed thick coverage of defects and focal radioopacity within defect area, but non-treated animals showed only fibrous healing. Histologically, small amount of new appositional bone growth was only seen at the edge of the defect at 6th week in Group III, but in Group IV, there are substantial amounts of new immature bone with well-organized patterns within the defect at 12th week postoperatively. In the radiodensitometric analysis, there was no statistically significant difference between Group I and Group III at 6th week. Compared with Group II, however, Group IV showed significant bony healing (p=0.0225) at 12th week. In conclusion, the fibrin sealant appeared to be an effective mediator in bone regeneration of this critical-sized calvarial defect model, but long-term implantation period should be needed for optimal results.

"Fast-tracking" in patients undergoing coronary artery surgery with severely impaired left ventricular function.

Cost containment and efficient resource utilization are motivating anaesthesiologists to early tracheal extubation of cardiac surgical patients. Fast track cardiac anaesthesia is both safe and cost-effective. We studied 45 patients with LVEF = 40% who underwent coronary revascularization and were put on the same fast track principle as those with normal ventricular function. Of these 31 patients (68.8%) had recent (<2 weeks) cardiac events requiring hospitalization. Acute myocardial infarction was documented in 8 patients (17.7%) and 23 (51.1%) had unstable angina. Congestive failure was present in 13 (28.9%) patients. Preoperative intravenous pharmacological support was needed in 17 (37.7%) patients, mechanical ventilation in 2 (4.5%) and IABP support in 1 (2.3%) patient. Anaesthetic management of these patients hinged on reduced usage of narcotics and muscle relaxants to facilitate early extubation. Operative strategy adopted was that of optimal revascularization. Beating heart surgery was adopted particularly in patients with low ejection fraction to avoid the attendant systemic and myocardial ill effects of cardiopulmonary bypass (CPB), especially in those who have had recent myocardial insults. Coronary surgery was performed on beating heart in 18 (40%) patients, while the other 27 (60%) patients were revascularised on CPB support. Of the 44 operative survivors, early extubation (<4 hours) was performed in 37 (84%) patients. Only 7 (16%) patients required ventilation for more than 24 hours. 3 out of these 7 patients were ventilated due to pulmonary reason and other 4 were ventilated since they were on IABP support. ICU stay was less than 24 hrs for 34/42 (80.9%) and hospital stay less than 6 days in 24/40 (60%). Fast tracking in cardiac surgery can be achieved with safety even in patients with impaired LV function.

Complications in 1000 consecutive treadmill tests.

Complication in 1000 consecutive Tread Mill Tests (TMT) done at the Cardiology Centre of Command Hospital (SC) Pune are reported. The tests were done following Bruce Protocol and analysed based on Selzer's criteria. Complications were noted in 18 cases (1.8%) which included ventricular fibrillation, ventricular tachycardia, atrial fibrillation, malignant VPBs, conduction disturbances, asystole following hyperventilation (HV), complete SA block following HV, atrial flutter following HV, hypotension and severe angina requiring ICCU care. No acute myocardial infarctions or deaths were noted. Coronary arteriography (CART) was done in 50 (5.0%) selected cases including 10 with complications. It is concluded that TMT is a safe procedure if carried out after proper patient selection and under supervision of an experienced and efficient team.