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El hiperparatiroidismo primario (HP) es una conocida endocrinopatía. Hace unos años la hipercalcemia moderada o grave junto con la afectación renal u ósea eran su forma más frecuente de presentación; sin embargo, actualmente la mayoría de los pacientes son asintomáticos y la hipercalcemia, discreta. La última conferencia NIH de consenso sobre el manejo del HP estableció algunos criterios sobre su manejo. Las neoplasia endocrinas múltiples (MEN) tipo 1 y 2 son síndrome genéticos causados por diversas alteraciones moleculares. El HP es la manifestación más frecuente de la MEN1 y la última en aparecer en la MEN2A. El carcinoma de paratiroides (CP) es un tumor infrecuente causante de hipercalcemia vía secreción de PTH. El presente artículo revisa el tratamiento del HP dentro de las MEN así como del CP. Además, se valora un nuevo tipo de agentes denominados “calcimiméticos” que pueden ser muy útiles en el tratamiento de ambas entidades.
Palabras clave:
Hiperparatiroidismo Primario
Neoplasia endocrina múltiple
Carcinoma de paratiroides
Calcimiméticos
Primary hyperparathyroidism (PHP) is a known endocrine disorder. Many years ago, the most frequent forms of clinical presentation were symptomatic renal or skeletal disease with moderate or severe hypercalcemia; however, currently, most patients have few symptoms and mild hypercalcemia. The last NIH Workshop on Asymptomatic PHP developed criteria for the management of this disease. Multiple endocrine neoplasia (MEN) types 1 and 2 are two genetic syndromes caused by different types of molecular abnormalities. PHP is the most common manifestation of MEN-1 and is the last feature to appear in MEN 2A. Parathyroid carcinoma (PC) is a rare neoplasm and an uncommon cause of parathyroid hormone (PTH)-dependent hypercalcemia. In this report, the treatment of PHP in MEN syndrome and PC are reviewed. Special attention is paid to a new class of drugs called “calcimimetics”, which are powerful compounds that may be highly useful in the treatment of both conditions.
Key words:
Primary hyperparathyroidism
Multiple endocrine neoplasia
Parathyroid carcinoma
Calcimimetics
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