Development and Validation of a Prediction Model and Score for Transthyretin Cardiac Amyloidosis Diagnosis: T-Amylo.

BACKGROUND: Although transthyretin cardiac amyloidosis (ATTR-CA) is often underdiagnosed, clinical suspicion is essential for early diagnosis. OBJECTIVES: The aim of this study was to develop and validate a feasible prediction model and score to facilitate the diagnosis of ATTR-CA. METHODS: This retrospective multicenter study enrolled consecutive patients who underwent 99mTc-DPD scintigraphy for suspected ATTR-CA. ATTR-CA was diagnosed if Grade 2 or 3 cardiac uptake was evidenced on 99mTc-DPD scintigraphy in the absence of a detectable monoclonal component or by demonstration of amyloid by biopsy. A prediction model for ATTR-CA diagnosis was developed in a derivation sample of 227 patients from 2 centers using multivariable logistic regression with clinical, electrocardiography, analytical, and transthoracic echocardiography variables. A simplified score was also created. Both of them were validated in an external cohort (n = 895) from 11 centers. RESULTS: The obtained prediction model combined age, gender, carpal tunnel syndrome, interventricular septum in diastole thickness, and low QRS interval voltages, with an area under the curve (AUC) of 0.92. The score had an AUC of 0.86. Both the T-Amylo prediction model and the score showed a good performance in the validation sample (ie, AUC: 0.84 and 0.82, respectively). They were tested in 3 clinical scenarios of the validation cohort: 1) hypertensive cardiomyopathy (n = 327); 2) severe aortic stenosis (n = 105); and 3) heart failure with preserved ejection fraction (n = 604), all with good diagnostic accuracy. CONCLUSIONS: The T-Amylo is a simple prediction model that improves the prediction of ATTR-CA diagnosis in patients with suspected ATTR-CA.

Long-term follow-up of differentiated thyroid carcinoma in children and adolescents.

Objectives The aims were to analyze the clinical features, response to treatment, prognostic factors and long-term follow-up of children and adolescents with differentiated thyroid carcinoma (DTC). Methods Eighty patients with DTC were studied retrospectively. All underwent total or near-total thyroidectomy, and in 75 cases, ablative iodine therapy was recommended. Patients were assessed periodically by tests for serum thyroglobulin levels and whole-body iodine scans. Age, gender, initial clinical presentation, histology, tumor stage, postoperative complications, radioiodine treatment protocol, treatment response, thyroglobulin (Tg), recurrence and long-term disease progression were evaluated. Results Seventy patients completed >2 years of follow-up (23 males, 47 females; median age: 14 years; range: 3-18 years). Sixty-two patients showed papillary DTC and eight, follicular DTC. Sixty-five percent presented nodal metastasis and 16%, pulmonary metastasis at diagnosis. Six months after first radioiodine treatment, 36.2% of patients were free of disease. Seven recurrences were documented. At the end of follow-up, overall survival was 100%, and 87.2% of patients were in complete remission. Nine patients had persistent disease. We found a significant association between stage 4 and persistent disease. Hundred percent of patients with negative Tg values at 6 months posttreatment were documented free of disease at the end of the follow-up. The analysis of disease-free survival based on radioiodine treatment protocols used showed no statistically significant differences. Conclusions DTC in children and adolescents is frequently associated with presence of advanced disease at diagnosis. Despite this, complete remission was documented after treatment in most cases, with a good prognosis in the long-term follow-up. Negative posttreatment thyroglobulin and stage 4 at diagnosis were significant prognostic variables.

Bone scintigraphy and radiolabeled white blood cell scintigraphy for the diagnosis of mandibular osteomyelitis.

The aim of this study was to evaluate the diagnostic efficacy of bone scintigraphy (BS) and radiolabeled white blood cell scintigraphy (WBCS) in detecting septic activity in the flat bones of the jaw. A retrospective analysis was conducted using 38 studies of combined BS plus WBCS: 33 of them 3-phase BS and 36 of them 2-phase WBCS. These studies were performed on 34 patients, 19 women and 15 men with a mean age of 56 years (22-79), who presented with suspected mandibular osteomyelitis, either acute or chronic exacerbation. The results were compared with histologic findings (55%) or with a minimum clinical/radiologic follow-up of 6 months (average, 21 months), when biopsy results were not available. BS showed a sensitivity of 100%, a specificity of 6.7%, a positive predictive value of 62%, and a negative predictive value of 100%. For WBCS, the corresponding values were as follows: 73.7%, 78.6%, 82%, and 69%. Accuracy was 63.2% for BS and 94.7% for WBCS. WBCS has proven to be a useful test for detecting septic activity in the jaw bone, being more effective than BS alone, which under certain circumstances, can return a very high false-positive rate.

Carcinoma diferenciado de tiroides en niños: estudio de 80 casos / Differentiated thyroid carcinoma in children: study of 80 cases

Fundamento y objetivo: Analizar el perfil clínico del carcinoma diferenciado de tiroides (CDT) y su relación con el pronóstico de la enfermedad. Pacientes y método: Se realizó un análisis retrospectivo de 80 niños con CDT. A todos se les practicó una tiroidectomía total y subtotal. Setenta y cinco niños recibieron dosis de yodo-131. Todos se controlaron anualmente mediante seguimiento clínico, analítico y pruebas de imagen. Resultados: De los 80 pacientes, 52 eran mujeres (edad media de 13,43 y desviación estándar [DE] de 3,6 años). El 87,5% presentó un aumento del diámetro cervical como primer síntoma, que correspondía en un 65% a un nódulo tiroideo y era más frecuente en el sexo femenino. La forma histológica papilar (84%) se presentó más frecuentemente que la folicular y se asoció a mayor presencia de adenopatías y metástasis. El 56,4% de los pacientes presentó enfermedad avanzada en el momento del diagnóstico. Nueve pacientes tenían antecedentes de irradiación previa. Un 32,5% de los pacientes presentó complicaciones posquirúrgicas. Al final del seguimiento (media de 10,79; DE de 5,69 años) sólo 9 pacientes persistían con enfermedad, variable asociada significativamente con el estadio 4. Conclusiones: El CDT en niños se presenta con mayor incidencia en el sexo femenino. La forma de inicio habitual es la presencia de un nódulo cervical. El tipo histológico predominante es el papilar, con elevada frecuencia, en forma de diseminación ganglionar y metastásica (AU)

Background and objective: The objective was to analyze the clinical profile of Differentiated Thyroid Carcinoma in children (DTC) and the predisposing factors to suffering the disease. Material and method: Eighty children with DTC were studied retrospectively. They all underwent total/near total thyroidectomy and 75 cases underwent ablative iodine therapy. Patients were controlled periodically with clinical, laboratory and imaging tests follow-up. Results: Twenty eight patients were male and 52 female (mean age: 13.43±3.6y). The 87.5% of patients had an increased cervical perimeter as the first clinical symptom, 65% of them corresponding to a thyroid nodule with a predominance of females. The papillary histological pattern was more frequent than the follicular pattern, and it was associated with the presence of lymph involvement and metastasis. About 56.4% of patients showed advanced disease at the time of diagnosis. 9 patients had previous irradiation. Surgical complications appeared in 32.5% of patients. At the end of follow-up (mean: 10.79±5.69y) 9 patients had persistent disease with a significant relation with stage 4. Conclusions: DTC presents a higher incidence in females than in males. Cervical node is the most frequent form of initial presentation. The papillary type is more prevalent than the follicular type, and it is frequently associated with lymph node involvement and metastatic spread (AU)

[Differentiated thyroid carcinoma in children: study of 80 cases]. / Carcinoma diferenciado de tiroides en niños: estudio de 80 casos.

BACKGROUND AND OBJECTIVE: The objective was to analyze the clinical profile of Differentiated Thyroid Carcinoma in children (DTC) and the predisposing factors to suffering the disease. MATERIAL AND METHOD: Eighty children with DTC were studied retrospectively. They all underwent total/near total thyroidectomy and 75 cases underwent ablative iodine therapy. Patients were controlled periodically with clinical, laboratory and imaging tests follow-up. RESULTS: Twenty eight patients were male and 52 female (mean age: 13.43+/-3.6 y). The 87.5% of patients had an increased cervical perimeter as the first clinical symptom, 65% of them corresponding to a thyroid nodule with a predominance of females. The papillary histological pattern was more frequent than the follicular pattern, and it was associated with the presence of lymph involvement and metastasis. About 56.4% of patients showed advanced disease at the time of diagnosis. 9 patients had previous irradiation. Surgical complications appeared in 32.5% of patients. At the end of follow-up (mean: 10.79+/-5.69 y) 9 patients had persistent disease with a significant relation with stage 4. CONCLUSIONS: DTC presents a higher incidence in females than in males. Cervical node is the most frequent form of initial presentation. The papillary type is more prevalent than the follicular type, and it is frequently associated with lymph node involvement and metastatic spread.