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A propósito de un caso de neoplasia endocrina múltiple tipo 1. Revisión de algunas manifestaciones clínicas y controversias en el tratamiento

The rare hereditary syndrome, multiple endocrine neoplasia type1 (MEN-1), is known to predispose affected individuals to endocrine neoplasms in a variety of tissues such as the parathyroid glands, the pituitary gland and the gastrointestinal tract. We describe the case of a man with traditionally-described manifestations (hyperparathyroidism and gastrinoma) and with other tumoral lesions arising from endocrine cells (insulinoma, gastric carcinoid, adrenal adenoma and pancreatic non-functioning neuroendocrine tumors) and non-endocrine cells (lipoma and collagenoma). Frequent recurrences in susceptible tissues that are not totally removed (as occurs in hyperparathyroidism and duodenal gastrinoma) and their unknown clinical significance have aroused current controversies in the therapeutic management of these entities, which is briefly reviewed.

Alteraciones endocrino-metabólicas inducidas por bexaroteno en el tratamiento del linfoma cutáneo de células T

Introduccion La utilizacion de bexaroteno, agonista selectivo del receptor de retinoide X (RXR), en pacientes con linfoma cutaneo de celulas T (CTLC), ha puesto de manifiesto la aparicion de dos complicaciones frecuentes, como son el hipotiroidismo central y la hipertrigliceridemia. Nos proponemos comprobar las repercusiones en las hormonas tiroideas y los lipidos plasmaticos en un pequeno grupo de pacientes con CTLC tratados con bexaroteno. Pacientes y metodo Presentamos a 6 pacientes (2 mujeres, 4 varones) con CTLC, en distintos estadios de la enfermedad y resistentes por lo menos a un tratamiento sistemico previo, que recibieron tratamiento con bexaroteno a dosis de 300 mg/m 2 . Se evaluo la TSH basal y la T4 libre y los lipidos plasmaticos (colesterol y trigliceridos) al inicio del tratamiento, a las 4 y las 8 semanas y, en los pacientes en que se suspendio el tratamiento, 4 semanas despues de finalizado. Resultados En 4 de 6 pacientes, la funcion tiroidea antes del tratamiento con bexaroteno era normal, en un paciente se desconocia y otro habia sido diagnosticado previamente de hipotiroidismo primario autoinmunitario subclinico. A las 4 semanas de iniciado el tratamiento, todos mostraron una disminucion de las concentraciones de tirotropina (TSH) y tiroxina (LT4), y 4 precisaron tratamiento con dosis sustitutivas de levotiroxina. Todos los pacientes presentaron elevaciones en las concentraciones plasmaticas de colesterol y/o trigliceridos que requirieron tratamiento con estatinas o fenofibrato. En aquellos en que se suspendio el bexaroteno, las hormonas tiroideas y los lipidos retornaron a cifras similares a las basales. Conclusiones El tratamiento con bexaroteno en pacientes con CTLC induce la aparicion de alteraciones endocrino-metabolicas, como hipotiroidismo central y dislipemia mixta, que requieren tratamiento con dosis sustitutivas de levotiroxina y farmacos hipolipemiantes en la mayoria de las ocasiones. Dichas alteraciones son reversibles tras la suspension del farmaco.

Medical nutrition therapy for gestational diabetes mellitus based on Mediterranean Diet principles: a subanalysis of the St Carlos GDM Prevention Study

Objectives To assess whether Mediterranean Diet (MedDiet)-based medical nutrition therapy facilitates near-normoglycemia in women with gestational diabetes mellitus (GDMw) and observe the effects on adverse pregnancy outcomes. Research design and methods This is a secondary analysis of the St Carlos GDM Prevention Study, conducted between January and December 2015 in Hospital Clínico San Carlos (Madrid, Spain). One thousand consecutive women with normoglycemia were included before 12 gestational weeks (GWs), with 874 included in the final analysis. Of these, 177 women were diagnosed with gestational diabetes mellitus (GDM) and 697 had normal glucose tolerance. All GDMw received MedDiet-based medical nutrition therapy with a recommended daily extra virgin olive oil intake ≥40 mL and a daily handful of nuts. The primary goal was comparison of hemoglobin A1c (HbA1c) levels at 36–38 GWs in GDMw and women with normal glucose tolerance (NGTw). Results GDMw as compared with NGTw had higher HbA1c levels at 24–28 GWs (5.1%±0.3% (32±0.9 mmol/mol) vs 4.9%±0.3% (30±0.9 mmol/mol), p=0.001). At 36–38 GWs values were similar between the groups. Similarly, fasting serum insulin and homeostatic model assessment insulin resitance (HOMA-IR) were higher in GDMw at 24–28 GWs (p=0.001) but became similar at 36–38 GWs. 26.6% of GDMw required insulin for glycemic control. GDMw compared with NGTw had higher rates of insufficient weight gain (39.5% vs 22.0%, p=0.001), small for gestational age (6.8% vs 2.6%, p=0.009), and neonatal intensive care unit admission (5.6% vs 1.7%, p=0.006). The rates of macrosomia, large for gestational age, pregnancy-induced hypertensive disorders, prematurity and cesarean sections were comparable with NGTw. Conclusions Using a MedDiet-based medical nutrition therapy as part of GDM management is associated with achievement of near-normoglycemia, subsequently making most pregnancy outcomes similar to those of NGTw.

About a case of multiple endocrine neoplasia type 1. Review of some clinical manifestations and treatment controversies

Abstract Multiple endocrine neoplasia type 1 (MEN1) is a rare hereditary syndrome known to predispose subjects to endocrine neoplasms in a variety of tissues such as the parathyroid glands, the pituitary gland, and the gastrointestinal tract. We report the case of a male patient who, in addition to the traditionally described conditions (hyperparathyroidism and gastrinoma), was found to have other tumor lesions arising from both endocrine cells (insulinoma, gastric carcinoid, adrenal adenoma, and non-functional pancreatic neuroendocrine tumors), and non-endocrine cells (lipoma and collagenoma). The frequent recurrence of lesions in not completely resected susceptible tissues (as occurs in hyperparathyroidism and duodenal gastrinoma) as well as doubts concerning their clinical significance in MEN1 has raised some questions regarding the therapeutic management of such lesions, and this controversy is briefly reviewed.

Evolución del hiperparatiroidismo primario en 56 pacientes. Comparación entre hiperparatiroidismo hipercalcémico y normocalcémico

Introduccion Actualmente, el hiperparatiroidismo primario (HPP) se considera una enfermedad escasamente sintomatica y poco progresiva. El HPP normocalcemico (HPPN) es una entidad reconocida e identificada esencialmente por complicaciones asociadas al HPP (osteoporosis y urolitiasis). Pacientes y metodo Estudio de 56 pacientes con HPP no operados o con seguimiento minimo de 1 ano previo a la paratiroidectomia. Se subdividio a los pacientes en HPP hipercalcemico (HPPH) (27 pacientes) y HPPN (29 pacientes). Se pretendio analizar las comorbilidades hipertension arterial [HTA] y nefrolitiasis) en el grupo total y en cada subgrupo, valorar la evolucion anual de calcemia, fosfatemia, paratirina PTH) y fosfatasa alcalina sericas, calciuria, filtrado glomerular y densidad mineral osea lumbar durante un periodo de 1 a 5 anos y hallar posibles diferencias entre HPPH y HPPN para estas variables. Resultados El 55,4% de los pacientes presentaban HTA y se registraron episodios de nefrolitiasis en el 37,5% de los casos. No se apreciaron cambios a lo largo del tiempo en los parametros bioquimicos y densitometricos ni en el grupo total ni por subgrupos. Excepto en los valores de calcio, fosforo y PTH sericos no se hallaron diferencias entre HPPH y HPPN. Conclusiones El HPP parece una enfermedad poco progresiva si bien no se incluyo en este estudio a los pacientes remitidos precozmente a cirugia con seguimiento previo escaso que podrian corresponder a casos mas agresivos. El HPPN es una entidad asociada a complicaciones similares a las del HPPH por lo que pensamos deberia someterse a los mismos criterios de derivacion quirurgica.