Evaluación de la densidad mineral ósea y de los parámetros de 3D-Shaper en la hipofosfatasia congénita del adulto / Agitated/inattentive children: explanatory models

Objetivo: Evaluar la densidad mineral ósea (DMO) y parámetros de 3D-Shaper a nivel de fémur proximal (FP) en adultos con hipofosfatasia (HPP) confirmada genéticamente y compararlos en aquellos sujetos con y sin fracturas. Material y métodos: Análisis transversal de datos densitométricos y de arquitectura ósea de la visita basal de un estudio longitudinal en el que se incluyeron pacientes con HPP. Se realizó un estudio densitométrico (Lunar Prodigy, GE iDXA) en FP y se empleó el software 3D-Shaper (version 2,7. Galgo Medical). Resultados: Se incluyeron 33 adultos con HPP con mutaciones en heterocigosis. Un 63,6% (21/33) fueron mujeres (42,9% postmenopáusicas), y 8 de los varones (66,6%) fueron mayores de 50 años. La media de edad fue 50,56±15,08 años, el 30,3% (10/33) tuvieron fracturas previas traumáticas, y un 15,2% (5/33), de estrés. La prevalencia de osteoporosis en CF fue del 11,8% (2/17) y de osteopenia, 82,4% (14/17). En premenopáusicas y varones jóvenes se detectó baja masa ósea para la edad en un 12,5% (2/16). Al comparar sujetos con fracturas de estrés y sin ellas, así como con traumáticas, no hubo diferencias en DMO. El 3D-Shaper mostró disminución del grosor cortical (mm) en pacientes con fracturas de estrés [1,8 (1,77-1,89)] frente a sujetos sin ellas [1,94 (1,87-2,03, p=0,03)] y en comparación con los que tuvieron fracturas traumáticas [1,97 (1,88-2,04), p=0,03]. Conclusión: Estos datos reflejan una discreta repercusión densitométrica en formas más leves del adulto. Estudios de arquitectura ósea pudieran resultar de interés para determinar pacientes susceptibles de presentar fracturas de estrés. (AU)

Objetivo: To evaluate bone mineral density (BMD) and 3D-Shaper parameters at the proximal femur (FP) level in adults with genetically confirmed hypophosphatasia (HPP) and to compare them in those subjects with and without fractures. Material and methods: Crosssectional analysis of densitometric data and bone architecture from the baseline visit of a longitudinal study in which patients with HPP were included. A densitometric study (Lunar Prodigy, GE iDXA) was carried out in FP using 3D-Shaper software (version 2.7. Galgo Medical). Results: 33 adults with HPP with heterozygous mutations were included. 63.6% (21/33) were women (42.9% postmenopausal), and 8 of the men (66.6%) were older than 50 years. The mean age was 50.56±15.08 years, 30.3% (10/33) had previous traumatic fractures and 15.2% (5/33) presented stress fractures. The prevalence of osteoporosis in CF was 11.8% (2/17) and of osteopenia, 82.4% (14/17). In premenopausal women and young men, low bone mass was detected for age in 12.5% (2/16). When comparing subjects with and without stress fractures, as well as traumatic ones, there were no differences in BMD. The 3D-Shaper showed a decrease in cortical thickness (mm) in patients with stress fractures [1.8 (1.77-1.89)] compared to subjects without them [1.94 (1.87-2.03, p=0.03)] and compared to those with traumatic fractures [1.97 (1.88-2.04), p=0.03]. Conclusions: These data reflect a discrete densitometric impact in milder forms of the adult. Bone architecture studies could be of interest in determining patients susceptible to stress fractures. (AU)

Unexpected primary bone lymphoma / Linfoma óseo primario inesperado

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Hallazgo casual de un timoma en un estudio de perfusión miocárdica con 99mTc-tetrofosmin / No disponible

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[Metastatic gastroenteropancreatic neuroendocrine tumours: 111In-pentetreotide and PET-FDG imaging]. / Tumor neuroendocrino del sistema gastroenteropancreático metastásico: imagen con 111In-pentetreótido y PET-FDG.

We present a patient with previous history of neuroendocrine tumour in the head of the pancreas, well differentiated and treated surgically. Two years later, there was suspicion of relapse. An endoscopy, CT scan and 111In-pentetreotide scintigraphy were performed. The 4 hour image showed two uptake lesions in the liver and the peripancreatic region. However, these focal uptakes had disappeared in the 24 hour image. In view of this result, a PET-FDG scan was carried out, showing two hypermetabolic lesions. The biodistribution of 111In-pentetreotide may be influenced by various parameters, including the time of scanning, and may show neuroendocrine tumours without somatostatin receptors in relapses. PET-FDG is an interesting tool to help characterise these cases.

Tumor neuroendocrino del sistema gastroenteropancreático metastásico: imagen con 111In-pentetreótido y PET-FDG / Metastatic gastroenteropancreatic neuroendocrine tumours: 111in-pentetreotide and PET-FDG imaging

Presentamos el caso de una paciente con el antecedente de un tumor neuroendocrino de la cabeza del páncreas, bien diferenciado, que es tratada mediante cirugía. Dos años después, ante la sospecha clínica de recidiva se le practica una endoscopia, una TAC y un rastreo con 111In-pentetreótido. En la imagen de las 4 horas se observaron lesiones con receptores de somatostatina en el hígado y en la región peripancreática. No obstante, esta captación desaparece a las 24 horas. Ante este hallazgo se realiza una PET-FDG mostrando focos de elevado metabolismo en ambas lesiones. La biodistribución del 111In-pentetreótido puede ser variable en el tiempo, y ser captada por tumores sin expresión previa de receptores de somatostatina en las recidivas. La PET-FDG puede ayudar en estos casos (AU)

We present a patient with previous history of neuroendocrine tumour in the head of the pancreas, well differentiated and treated surgically. Two years later, there was suspicion of relapse. An endoscopy, CT scan and 111In-pentetreotide scintigraphy were performed. The 4 hour image showed two uptake lesions in the liver and the peripancreatic region. However, these focal uptakes had disappeared in the 24 hour image. In view of this result, a PET-FDG scan was carried out, showing two hypermetabolic lesions. The biodistribution of 111In-pentetreotide may be influenced by various parameters, including the time of scanning, and may show neuroendocrine tumours without somatostatin receptors in relapses. PET-FDG is an interesting tool to help characterise these cases (AU)

[Gammagraphic study of nodular adrenal hyperplasia]. / Estudio gammagráfico de la hiperplasia suprarrenal nodular.

A common cause of the Cushing's Syndrome (CS) is nodular suprarenal hyperplasia (NSH), which is characterized by the presence of nodes in both suprarenal glands. Its pathophysiology is not well known and its diagnosis is quite difficult due to the heterogenicity of the biochemical and radiological data. We analyzed the suprarenal gammagraphies (SRG) of 7 patients with anatomopathological diagnosis of NSH. Bilateral uptake was observed in all cases and in five patients, such bilateral uptake presented a clear asymmetry. We believe that these observations demonstrate a bilateral suprarenal affectation and are of great use in order to orientate the diagnosis of NSH and, especially, in order to differentiate it from other suprarenal diseases causing CS, like adenomas, in which uptake is unilateral.