INTRODUCTION Newborns in need of surgery are a challenge to manage and require highly specialized centers with multidisciplinary surgical teams. Since the 1980s, neonatal surgical survival has increased by up to 70% in some countries, mainly due to advances in neonatal intensive care, anesthesia and surgical technique.

OBJECTIVE Describe surgical case fatality and survival in a neonatal reference hospital in Cuba, estimate risk of death, and identify potential risk predictors among neonatal characteristics.

METHODS A retrospective cohort study was conducted based on hospital administrative data and clinical records for a series of surgical cases in the neonatal intensive care unit of Havana’s William Soler University Children’s Hospital from January 2005 to December 2015. All neonates who underwent surgery during the study period were included. The dependent variable was discharge status (alive, deceased); independent variables were: sex, age (in days) at time of surgery, gestational age, birth weight, indication for surgery, surgical order (first, repeat), and presence of sepsis or other postoperative complications. The study used contingency tables to analyze associations between neonatal characteristics and discharge status. A classification tree was used to obtain simple estimates of surgical risk.

RESULTS Survival was 91.3% (675/739) among 739 neonates who underwent surgery. The majority were male (58.7%, 434/739), full term (84.2%, 622/739), and of normal birth weight (80.6%, 596/739). Most surgeries were performed in the first 10 days of life. Digestive system anomalies constituted the most common surgical indication (57.6%, 426/739); among these anorectal malformations (26.8%, 114/426) and esophageal atresia (17.4%, 74/426) predominated. Survival rates were lower for digestive perforation (57.7%, 15/26), gastroschisis (57.1%, 4/7) and intestinal atresia (73%, 27/37). Neonates in the youngest and oldest age groups at time of surgery faced highest risk of death, especially those in the older group. Term infants with normal birth weight operated on for the first time had survival rates >95%.

CONCLUSIONS Survival was high and low birth weight conferred the worst prognosis. Infants with normal birth weight operated on for the first time had the greatest probability of survival.

KEYWORDS Neonatal surgery, intensive care, neonatal; congenital disorders, birth defects, congenital abnormalities, surgery, surgery, mortality, case fatality, Cuba

INTRODUCCIÓN La insuficiencia hepática aguda es una enfermedad poco frecuente en pacientes pediátricos y es una de las emergencias médicas más desafiantes debido a su pronóstico y a sus implicaciones terapéuticas.  Los mejores resultados globales provienen de estudios multicéntricos en países desarrollados.  En Cuba, no hay estudios anteriores sobre esta enfermedad en niños.

OBJETIVOS Se describen las principales características clínicas de niños cubanos tratados en un centro de referencia nacional para la insuficiencia hepática aguda, según la definición dada por los criterios de diagnóstico reconocidos para los pacientes pediátricos.

MÉTODOS Se realizó un estudio en una serie de casos que comprendía los pacientes diagnosticados con insuficiencia hepática aguda tratados entre 2005 y 2011 en el servicio de hepatología y trasplante hepático del Hospital Pediátrico Universitario William Soler en La Habana. Las variables fueron: grupo etario, etiología de la insuficiencia hepática aguda, grado de encefalopatía de origen hepático, variables de la química sanguínea y el resultado clínico (si se recuperaba o no espontáneamente  la función hepática). Se evaluaron las asociaciones entre las variables mediante las tablas de contingencia, y se calculó la tasa de letalidad como riesgo relativo con un intervalo de confianza de 95%. Las medias de los resultados de los análisis de laboratorio se compararon con la prueba U de Mann-Whitney.

RESULTADOS La mediana de la edad de los 31 pacientes estudiados (14 niños y 17 niñas) fue de 24 meses (con el rango de 1-180 meses). El tiempo entre el inicio de los síntomas y el diagnóstico de insuficiencia hepática aguda fue de 25.1 días (DE 16.8). La etiología más común fue la infección, que se encontró en el 61.3% de los casos (19/31); los virus no hepatotrópicos, especialmente el citomegalovirus, predominaron en niños pequeños. Hubo recuperación espontánea en 15 pacientes (48.4%), tres (9.7%) recibieron trasplantes y 13 fallecieron, para una tasa de letalidad de 41.9%.El resultado final no estuvo relacionado con la etiología (p = 0.106), pero tenía una relación estadística con el grado de encefalopatía de origen hepático(p <0.01), por lo que el 77.8% de los pacientes (7/9) con encefalopatía de grados III-IV fallecieron, para un riesgo relativo de 4.0 (IC 95% 1.15-13.8), frente al 11.1% (1/9) con encefalopatía de grado II o menos. Los niveles de colesterol fueron significativamente menores en pacientes que no se recuperaron espontáneamente (p <0.01).

CONCLUSIONES Las características clínicas de los pacientes en esta serie de casos fueron similares a los descritos en los países desarrollados. El hecho de que los virus no hepatotrópicos (básicamente el citomegalovirus en niños pequeños) son la causa principal de la insuficiencia hepática aguda en los niños cubanos, plantea la necesidad de realizar estudios epidemiológicos más profundos, e identificar los determinantes subyacentes de este fenómeno.

PALABRAS CLAVE Insuficiencia hepática aguda/etiología, trasplantes, infecciones, citomegalovirus, niños, pediatría, Cuba

INTRODUCTION Acute liver failure is rare in pediatric patients and is one of the most challenging medical emergencies due to its prognostic and therapeutic implications. The best scientific evidence worldwide comes from multicenter studies in developed countries. In Cuba, there are no prior studies of this disorder in children.

OBJECTIVES Describe the main clinical features of Cuban children treated at a national referral center for acute liver failure, as defined by recognized diagnostic criteria for pediatric patients.

METHODS A case series study was conducted comprising patients diagnosed with acute liver failure treated from 2005 to 2011 in the hepatology and liver transplant service at Havana’s William Soler University Children’s Hospital. Variables were age group, etiology of acute liver failure, grade of hepatic encephalopathy, blood chemistry variables, and clinical outcome (whether or not spontaneous recovery of liver function occurred). Associations between variables were assessed using contingency tables, and case fatality was calculated, as well as relative risk with its 95% confidence interval. The Mann-Whitney U test was used to compare means of laboratory test results.
RESULTS Median age of the 31 patients studied (14 boys and 17 girls) was 24 months (range 1–180). Time between symptom onset and diagnosis of acute liver failure was 25.1 days (SD 16.8). Infection was the most common etiology, present in 61.3% of cases (19/31); nonhepatotropic viruses, especially cytomegalovirus, predominated in infants. Spontaneous recovery occurred in 15 patients (48.4%), 3 (9.7%) received transplants, and 13 died, for a case fatality of 41.9%. Outcome was not associated with etiology (p = 0.106), but was statistically associated with degree of hepatic encephalopathy (p <0.01): 77.8% of patients (7/9) with grades III–IV encephalopathy died, for a relative risk of 4.0 (95% CI 1.15–13.8), versus 11.1% (1/9) with grade II or less encephalopathy. Cholesterol levels were significantly lower in patients who failed to recover spontaneously (p <0.01).

CONCLUSIONS Patients’ clinical characteristics in this case series were similar to those described in developed countries.The fact that nonhepatotropic viruses (basically, cytomegalovirus in infants) are the main cause of acute liver failure in Cuban children calls for further epidemiologic study and identification of local underlying determinants of this phenomenon.

KEYWORDS Acute liver failure/etiology, transplants, infections, cytomegalovirus, children, pediatrics, Cuba