RESUMEN
A register for medullary thyroid carcinoma (MTC) in FRG has been set up by the "German Medullary Thyroid Carcinoma Study Group" in 1988. The aim is to provide a basis for collaborative work on MTC especially in the hereditary forms (i.e. multiple endocrine neoplasia [MEN] type IIa, IIb). For these hereditary varieties reliable screening tests exist and, if the disease is detected by family screening in an early stage, curative surgery is possible. Until now 408 patients (234 female, 174 male) with MTC have been reported by 17 cooperative centers. The mean age at diagnosis was 45.5 years. 25% (n = 104) are hereditary forms, most of them MEN IIa (n = 86), 13 are MEN IIb and 18 belong to the familial variety without other endocrinopathy. The mean age at diagnosis for MEN IIa was 36.5 years, MEN IIb 26.9 and for the pure familial form 27.5 years. As 33 patients per year have been diagnosed since 1982, nearly 25% of all expected cases of MTC in FRG have been registered.
Asunto(s)
Carcinoma/epidemiología , Neoplasia Endocrina Múltiple/epidemiología , Sistema de Registros , Neoplasias de la Tiroides/epidemiología , Adulto , Anciano , Estudios Transversales , Femenino , Alemania Occidental/epidemiología , Humanos , Incidencia , Masculino , Persona de Mediana EdadAsunto(s)
Enfermedad de Hodgkin/tratamiento farmacológico , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Preescolar , Ensayos Clínicos como Asunto , Ciclofosfamida/administración & dosificación , Doxorrubicina/administración & dosificación , Alemania Occidental , Enfermedad de Hodgkin/diagnóstico , Humanos , Lactante , Recién Nacido , Prednisona/administración & dosificación , Procarbazina/administración & dosificación , Factores de Riesgo , Vincristina/administración & dosificaciónRESUMEN
We report on a case of autosomal dominant periodic ataxia. So far, fourteen families have been described with this rare disease. An early diagnosis is important; the patients can be treated effectively with acetazolamide.
Asunto(s)
Periodicidad , Degeneraciones Espinocerebelosas/genética , Acetazolamida/uso terapéutico , Niño , Aberraciones Cromosómicas/genética , Trastornos de los Cromosomas , Diagnóstico Diferencial , Genes Dominantes , Humanos , Masculino , Degeneraciones Espinocerebelosas/tratamiento farmacológicoRESUMEN
It was the objective of the DAL study HD-85 to examine the possibility of a reduction of chemotherapy, compared to the study HD-82 which had applied a combined treatment strategy resulting in a relapse rate of only 2.5% after 4 years, and a proportion of intercurrent deaths of 1.5%, in a total of 203 patients. Procarbazine was eliminated in the OPPA-therapy (OPA) and replaced by methotrexate in the COPP-cycles (COMP). Chemotherapy consisted in 2 cycles of OPA for stage I/IIA (group 1), 2 cycles of OPA and 2 cycles of COMP for stage IIB/IIIA (including IEA/IIEA) (group 2), and 2 cycles of OPA and 4 cycles of COMP for stage IIIB/IV (group 3). The subsequent radiotherapy was limited to the initially involved fields, the dosage of 35, 30 or 25 Gy depending on the extent of chemotherapy. Regions showing incomplete tumor regression after chemotherapy received another 5 Gy, in patients of groups 2 and 3. Between Jan. 1985 to Nov. 1986, 103 children with Hodgkin's disease from 42 participating hospitals were enrolled in this study. 98 patients were treated according to protocol (59 boys, 39 girls). A specific strategy for the selective indication of laparotomy and splenectomy was applied. With this strategy no laparotomy was performed in 39/98 patients (40%). 67 patients (68%) retained their spleen. 4 patients showed progression of the disease under chemotherapy, all others achieved remission. 16 patients relapsed before Dec. 31, 1987. So far no child of this study has died.(ABSTRACT TRUNCATED AT 250 WORDS)