Carcinoma papilar de tiroides en un niño con hipotiroidismo congénito dishormonogénico: Reporte de un caso / Papillary thyroid carcinoma in a child with congenital dyshormonogenetic hypothyroidism: Case report

Introducción: El cáncer papilar de tiroides (CPT) es una enfermedad infrecuente en pediatría. La presentación de CPT asociado a hipotiroidismo congénito (HC) dishormonogénico es excepcional, y hay pocos casos reportados en la literatura. Objetivo: Presentar un caso de CPT en un paciente con HC dishormonogénico sin bocio, expuesto a radiación ionizante. Evaluar asociaciones entre estos factores y el desarrollo de CPT. Caso clínico: Paciente varón con antecedentes de HC dishormonogénico, por lo que recibió suplementación precoz con levotiroxina, logrando niveles normales de tirotropinas y hormonas tiroideas. Con antecedentes de cardiopatía congénita, fue sometido tratamiento intervencional con 10 cateterismos cardíacos y aproximadamente 26 radiografías de tórax con dosis pediátrica. A la edad de 6 años se encontró un nódulo tiroideo mediante ecografía. La citología por punción aspirativa con aguja fina confirmó alta sospecha de carcinoma tiroideo (Bethesda 5). El estudio de etapificación no mostró metástasis en el tórax ni en el cerebro. Fue sometido a tiroidectomía total y el análisis histopatológico reveló un microcarcinoma papilar de 0,5 cm intratiroideo, sin evidencia de diseminación. Conclusión: Las mutaciones genéticas propias de esta enfermedad y la exposición a radiación ionizante pudieran estar implicadas en el desarrollo de CPT. Es probable que haya vías fisiopatológicas comunes que requieren mayor investigación.

Introduction: Papillary thyroid carcinoma (PTC) is a rare childhood disease. The development of PTC in dyshormonogenetic congenital hypothyroidism (CH) is infrequent, with very few case reports in literature. Objective: To report a case of PTC in a boy with dyshormonogenetic CH without goitre and exposed to ionising radiation. To evaluate relationships between these factors and development of PTC. Case report: We present a boy with dyshormonogenetic CH since birth. Early hormonal substitution was initiated, with subsequent normal levels of thyrotropin and thyroid hormones. He has also congenital cardiomyopathy, exposed to interventional treatment with 10 heart catheterisations, and approximately 26 chest X-rays at paediatric doses. A thyroid nodule was found in thyroid echography at the age of 6 years old. Fine needle aspiration biopsy confirmed high probability of thyroid carcinoma (Bethesda 5). The pre-surgical thorax and cerebral scan showed no evidence of metastasis. The patient underwent total thyroidectomy. Pathological examination revealed a 0.5 cm papillary thyroid micro-carcinoma in the right lobe, with no evidence of dissemination. Conclusion: Genetic mutations and radiation exposure may play an important role in the development of PTC. There may be common pathways between dyshormonogenetic CH and thyroid carcinoma that need further investigation.

[Papillary thyroid carcinoma in a child with congenital dyshormonogenetic hypothyroidism. Case report]. / Carcinoma papilar de tiroides en un niño con hipotiroidismo congénito dishormonogénico. Reporte de un caso.

INTRODUCTION: Papillary thyroid carcinoma (PTC) is a rare childhood disease. The development of PTC in dyshormonogenetic congenital hypothyroidism (CH) is infrequent, with very few case reports in literature. OBJECTIVE: To report a case of PTC in a boy with dyshormonogenetic CH without goitre and exposed to ionising radiation. To evaluate relationships between these factors and development of PTC. CASE REPORT: We present a boy with dyshormonogenetic CH since birth. Early hormonal substitution was initiated, with subsequent normal levels of thyrotropin and thyroid hormones. He has also congenital cardiomyopathy, exposed to interventional treatment with 10 heart catheterisations, and approximately 26 chest X-rays at paediatric doses. A thyroid nodule was found in thyroid echography at the age of 6 years old. Fine needle aspiration biopsy confirmed high probability of thyroid carcinoma (Bethesda 5). The pre-surgical thorax and cerebral scan showed no evidence of metastasis. The patient underwent total thyroidectomy. Pathological examination revealed a 0.5cm papillary thyroid micro-carcinoma in the right lobe, with no evidence of dissemination. CONCLUSION: Genetic mutations and radiation exposure may play an important role in the development of PTC. There may be common pathways between dyshormonogenetic CH and thyroid carcinoma that need further investigation.

GCS as a predictor of mortality in patients with traumatic inferior vena cava injuries: a retrospective review of 16 cases.

INTRODUCTION: Recent research has determined Glasgow Coma Scale (GCS) to be an independent predictor of mortality in patients with traumatic inferior vena cava (IVC) injuries. The aim of this study was to evaluate the use of GCS, as well as other factors previously described as determinants of mortality, in a cohort of patients presenting with traumatic IVC lesions. METHODS: A 7-year retrospective review was undertaken of all trauma patients presenting to a tertiary care trauma center with trauma related IVC lesions. Factors described in the literature as associated with mortality were assessed with univariate analysis. ANOVA analysis of variance was used to compare means for continuous variables; dichotomous variables were assessed with Fischer's exact test. Logistic regression was performed on significant variables to assess determinants of mortality. RESULTS: Sixteen patients with traumatic IVC injuries were identified, from January 2005 to December 2011. Six patients died (mortality, 37.5%); the mechanism of injury was blunt in one case (6.2%) and penetrating in the 15 others (93.7%). Seven patients underwent thoracotomy in the operating room (OR) to obtain vascular control (43.7%). Upon univariate analysis, non-survivors were significantly more likely than survivors to have lower mean arterial pressures (MAP) in the emergency room (ER) (45.6 +/- 8.6 vs. 76.5 +/- 25.4, p = 0.013), a lower GCS (8.1 +/- 4.1 vs. 14 +/- 2.8, p = 0.004), more severe injuries (ISS 60.3 +/- 3.5 vs 28.7 +/- 22.9, p = 0.0006), have undergone thoracotomy (83.3% vs. 16.6%, p = 0.024), and have a shorter operative time (105 +/- 59.8 min vs 189 +/- 65.3 min, p = 0.022). Logistic regression analysis revealed GCS as a significant inverse determinant of mortality (OR = 0.6, 0.46-0.95, p = 0.026). Other determinants of mortality by logistic regression were thoracotomy (OR = 20, 1.4-282.4, p = 0.027), and caval ligation as operative management (OR = 45, 2.28-885.6, p = 0.012). CONCLUSIONS: GCS, the need to undergo thoracotomy, and caval ligation as operative management are significant predictors of mortality in patients with traumatic IVC injuries.