Analysis of the morbidity of a rare disease using data from the Medical Information Systems Program (PMSI): the example of sickle cell disease

Introduction. As part of its surveillance of rare diseases in France, the InVS produces indicators of hospital morbidity based on the PMSI. Sickle cell disease is the first condition for which the progression of patients and hospital stays has been studied. This genetic hemoglobin disorder is one of the most common hereditary diseases in France. Methods. All hospital stays and short-stay visits for residents of metropolitan France and the French overseas departments (Martinique, Guadeloupe, Réunion), in whose summaries at least one ICD-10 code for sickle cell disease was coded as the principal diagnosis (PD), related diagnosis (RD), or associated diagnosis (AD), excluding sickle cell trait (a minor form of sickle cell disease). The analysis covers the period 2004–2009 and focused on the description of hospital stays and, after linkage, of patients. Results. Over the 2004–2009 period, 137,447 hospital stays by individuals with sickle cell disease were identified, of which 77% were for inpatient care and 23% for outpatient visits (primarily for transfusions), corresponding to approximately 7,355 patients treated annually. The hospitalization rate (excluding outpatient visits) is 28 per 100,000 inhabitants, and the number of hospitalizations increases by an average of 3.5% (CI: [1.7%; 5.2%]) per year. People with sickle cell disease are hospitalized an average of three times a year and accumulate nearly ten days of hospitalization over 12 months, with significant variations by age. The number of children under two years of age identified in the PMSI is comparable to those screened by the AFDPHE (an average of 375 cases per year between 2006 and 2009). Discussion and conclusion. The defined indicators do not allow for estimating the prevalence of the disease, but they do offer the possibility of tracking changes in the annual number of hospitalized patients and the characteristics of their hospital care. (R.A.)

Author(s): Paty AC, Suzan F

Publishing year: 2012

Pages: S30

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