RESUMEN
X-Linked hypophosphatemia (XLH) is the most common form of hereditary rickets caused by loss-of function mutations in the PHEX gene. XLH is characterized by hypophosphatemia secondary to renal phosphate wasting, inappropriately low concentrations of 1,25 dihydroxyvitamin D and high circulating levels of fibroblast growth factor 23 (FGF23). Short stature and rachitic osseous lesions are characteristic phenotypic findings of XLH although the severity of these manifestations is highly variable among patients. The degree of growth impairment is not dependent on the magnitude of hypophosphatemia or the extent of legs´ bowing and height is not normalized by chronic administration of phosphate supplements and 1α hydroxyvitamin D derivatives. Treatment with growth hormone accelerates longitudinal growth rate but there is still controversy regarding the potential risk of increasing bone deformities and body disproportion. Treatments aimed at blocking FGF23 action are promising, but information is lacking on the consequences of counteracting FGF23 during the growing period. This review summarizes current knowledge on phosphorus metabolism in XLH, presents updated information on XLH and growth, including the effects of FGF23 on epiphyseal growth plate of the Hyp mouse, an animal model of the disease, and discusses growth hormone and novel FGF23 related therapies.
Asunto(s)
Raquitismo Hipofosfatémico Familiar/metabolismo , Factores de Crecimiento de Fibroblastos/metabolismo , Trastornos del Crecimiento/metabolismo , Animales , Raquitismo Hipofosfatémico Familiar/complicaciones , Factor-23 de Crecimiento de Fibroblastos , Trastornos del Crecimiento/tratamiento farmacológico , Trastornos del Crecimiento/etiología , HumanosRESUMEN
No disponible
Asunto(s)
Humanos , Suplementos Dietéticos , Infecciones por VIH , Administración Oral , Hepatopatías , NeoplasiasRESUMEN
Se analizan los resultados de un estudio prospectivo, no randomizado, de 92 pacientes portadores de un cáncer prostático avanzado: 29 en etapa D1 y 63 en etapa D2, tratados con orquiectomía y quimioterapia adyuvante. Esta última consistió en 12 ciclos, cada 28 días, de 600 mg/m² de ciclosfosfamida y 600 mg/m² de 5-Fluoruracilo. La sobrevida a 5 años es 33.5 por ciento para toda la serie: 71.3 por ciento en etapa D1 y 27.5 por ciento en etapa D2. Se analiza además la respuesta a la quimioterapia y el período libre de progresión. Los resultados se comparan favorablemente con los del bloqueo androgénico total
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Quimioterapia Adyuvante , Ciclofosfamida/uso terapéutico , Fluorouracilo/uso terapéutico , Neoplasias de la Próstata/tratamiento farmacológico , Ciclofosfamida , Supervivencia sin Enfermedad , Fluorouracilo , Orquiectomía , Estudios Prospectivos , Neoplasias de la Próstata/cirugíaRESUMEN
Inhalant food allergy has been described many times in literature, but double-blind clinical trials to support successful hyposensitization to these allergens has seldom been reported. Some authors have suspected that certain adverse reactions after immunotherapy may be mediated by immune complexes. Furthermore, the FDA does not recommend injection therapy with food extracts. We present a study on the detection of adverse effects after immunotherapy with an inhalant food (wheat flour) in a double-blind clinical trial in 26 patients with bakers' asthma. We investigated the presence of circulating immune complexes (CICs) after 2 years of treatment with hyposensitization to wheat flour.