Systemic Lupus Erythematosus in Filipino children: A 10-year retrospective analysis of mortality, morbidity and survival

@#Background. Systemic Lupus Erythematosus (SLE) in children has been estimated to account for 15 to 20% of all SLE cases worldwide. It was described to have more severe disease at presentation including renal, neuropsychiatric, and hematologic involvements; more disease activity over time, and a significantly higher risk of organ damage. Thus, considered a significant risk factor for mortality among adult SLE patients. Objective. This is a retrospective cohort study aimed to determine the clinical profile, outcome, and survival of SLE among Filipino children. Methods. All SLE patients, less than 19 years old, diagnosed in the pediatric department of a tertiary hospital from January 2008 to December 2017 were included in the study. Their medical charts were retrieved for data gathering. Demographics, and clinical disease characteristics were collected from admission and on subsequent follow ups. Lost to follow up patients were contacted for updates of their current clinical status. Results. A total of 261 pediatric SLE patients were gathered. Average age at diagnosis is 14.5 years old (±2.7), with female to male ratio of 16:1. Symptoms starts at 3 months prior to consult (±2.1). Upon diagnosis, most of the patients have fever, malar rash, alopecia, oral ulcers, and proteinuria. Most common systemic organ involvement through time were mucocutaneous, hematologic, and renal. Steroids were the mainstay management for all patients, in which 95% started on oral Prednisone, while 71% needed IV Methylprednisolone in at least once during the disease course. Two patients received biologic treatment. Overall mortality rate was 14.9%, identified to be secondary to sepsis and/or SLE activity. Myocarditis, pleural effusion, and seizures were identified as significant risk factors for mortality. Survival rate at 1 year and 10 years were 92% and 79%, respectively. Conclusion. SLE in Filipino children mostly presents with mucocutaneous symptoms. Presence of seizures, myocarditis, and pleural effusion at any time of the disease entails risk for mortality. SLE nephritis is a substantial cause of morbidity due to its chronicity. The survival rate of Filipino children with SLE is comparable with the data from other developing countries.

Resultados inmediatos y alejados del switch arterial en pacientes condextrotransposición de grandes arterias: experiencia de 20 años / Early and late results of the switch operation for d-transposition of the great vessels. clinical experience in 108 patients operated on between 1992 and 2012

Objetivos: Comunicar los resultados de la operación de switch arterial en pacientes portadores de dextro Transposición de Grandes Arterias (D-TGA) y evaluar su evolución en el tiempo. Pacientes y Método: Estudio retrospectivo de pacientes sometidos a switch arterial entre mayo de 1992 y noviembre de 2012. Se comparó período 1 (1992 a 2002) con período 2 (2003 a 2012). Se definió D-TGA simple aquella sin lesiones asociadas y D-TGA compleja aquella con asociación de comunicación interventricular o coartación aórtica. Resultados: Un total de 108 pacientes componen la serie, 44 en el período 1 y 64 en el período 2, sin diferencias demográficas y anatómicas entre ambos períodos. Setenta tenían D-TGA simple y 38 D-TGA compleja. La mortalidad operatoria en el período 1 fue 33% versus 8,4 por ciento en el período 2 (p< 0.025); hubo tendencia a mayor mortalidad en pacientes con anatomía coronaria compleja. Se observó una disminución del riesgo relativo de mortalidad de 68,7 por ciento en el período 2. El 28,7 por ciento presentaron complicaciones postoperatorias, sin diferencias entre ambos períodos. Un paciente falleció en forma alejada. La mediana de seguimiento fue de 60 meses y la supervivencia de 84,25 por ciento a 10 y 20 años. Se realizaron 16 re intervenciones, principalmente plastías percutáneas de ramas pulmonares. La mayoría de los pacientes se mantenían asintomáticos. La mortalidad operatoria de los últimos 5 años fue 2,6 por ciento. Conclusiones: La mortalidad operatoria ha disminuido significativamente a lo largo de 20 años; persiste un mayor riesgo en ciertos patrones de anatomía coronaria. La supervivencia alejada y libre de reintervenciones es muy favorable.

Aim: to report the results of the arterial Switch operation in patients with D-transposition of the great vessels (D-TGA) and to evaluate their late course. Patients and Methods: A retrospective review of the clinical data on patients consecutively operated on for D-TGA using the switch procedure. Results obtained in patients operated on between 1992 and 2002 (Period 1) were compared to those obtained in patients undergoing their operation between 2013 and 2012. Patients with D-TGA and no complications were compared to those who had interventricular septal defect or aortic coarctation associated their TGA. Results: 44 patients belonged in Period 1 and 64 in Period 2 (total 108). Demographic and anatomical characteristics were similar in both periods. Simple D-TGA was present in 70 patients and complex D-TGA in 38. Operative mortality was higher in Period 1 compared to Period 2 (33 per cent vs. 8.4 percent, p<0.025). A higher, albeit not statiscally significant mortality was observed in patients with complex as opposed to simple D-TGA. A 68.7 per cent relative reduction mortality risk was observed in Period 2. Complications developed in 28.7 per cent of patients, with no difference between periods. Only 1 patient died during late follow up. Median follow up was 60 months and survival rate was 84.3 percent at 10 y 20 years. Sixteen patients required re-intervention, mainly to perform percutaneous plastic procedures on pulmonary artery branches. Most patients had an asymptomatic course during follow up. Surgical mortality for the last 5 years was 2.6 percent. Conclusion: surgical mortality for the arterial switch operation in patients with D-TGA has significantly decreased along a 20 year period. An increased surgical risk persists for patients with certain forms of coronary artery anomalies. Late survival free of re-intervention was the rule in these patients.