ABSTRACT
This study forms part of a research project seeking to develop a standardized questionnaire by which clinicians can assess the impact of growth hormone (GH) deficiency and its treatment on the "perceived health" or health-related quality of life of adults. The specific aim of this study was to translate and adapt for French patients the AGHDA (Adult Growth Hormone Deficiency Assessment) a standardized health-related quality of life measure for use with GH-deficient adults, initially developed in the United Kingdom, and to collect data which could be used to assess the main psychometric characteristics of its French version the ISPA-HC (Indicateur de Santé Perceptuelle Adulte-Hormone de Croissance). The main properties analyzed are: 1/ The scale's acceptability, as determined by means of face-to-face interviews with a small number of subjects, then by an ad hoc questionnaire administered during a test-retest study; 2/ The scale's reliability, as determined by a test-retest study (with a 15-days interval between tests); 3/ The scale's concurrent validity, as expressed by comparison with scores obtained by means of a generic quality of life scale, the ISPN (the French version of the Nottingham Health Profile). The results of this first trial with the ISPA-HC are conforming to what one can expect from a good instrument. The ISPA-HC has been shown to have very good levels of reliability and internal consistency. Its scores show a close correlation with those of the ISPN (the French version of the Nottingham Health Profile). This instrument can be used to measure variations in the perceived health of subjects with growth hormone deficiency. Its responsiveness to change is to be examined in subsequent studies.
Subject(s)
Human Growth Hormone/deficiency , Quality of Life , Surveys and Questionnaires , Adult , France , Health Status , Human Growth Hormone/therapeutic use , Humans , Pilot Projects , Reproducibility of ResultsABSTRACT
INTRODUCTION: Histiocytosis X or Langerhans cell granuloma is a rare disease characterised by the proliferation of phagocytes which have normal cell markers. EXEGESIS: We report the case of a 44-year old woman with hypothalamo-hypophyseal Langerhans cell histiocytosis with genital and oral cutaneo-mucous involvements. After several non-active treatments, thalidomide began. Oral and genital lesions healed quickly. Recurrence of symptoms was observed six months after the treatment was stopped and a complete remission was obtained when thalidomide was reintroduced. The regression of hypothalam-hypophyseal granuloma infiltration was confirmed by MRI and the treatment was well tolerated by the patient. CONCLUSION: Cutaneo-mucous Langerhans cell disease as well as hypothalamo-hypophyseal histiocytosis may both benefit from a treatment with thalidomide.
Subject(s)
Genital Diseases, Female/etiology , Histiocytosis, Langerhans-Cell/drug therapy , Hypothalamic Diseases/etiology , Immunosuppressive Agents/pharmacology , Mouth Diseases/etiology , Mouth Mucosa/pathology , Pituitary Diseases/etiology , Thalidomide/pharmacology , Adult , Female , Genital Diseases, Female/drug therapy , Histiocytosis, Langerhans-Cell/complications , Humans , Hypothalamic Diseases/drug therapy , Magnetic Resonance Imaging , Mouth Diseases/drug therapy , Pituitary Diseases/drug therapy , Skin Diseases/drug therapy , Skin Diseases/etiologyABSTRACT
The authors report a simultaneous observation of Biermer's anaemia and myeloma in a 77 year old woman. There are now 22 cases known to this association. A relationship between the two immunological diseases was suggested but the authors think that there is no proof of such a relationship.
