Rare Diseases Symptoms Automatic Extraction

[National consensus regarding the prescription of inhaled corticosteroids in cystic fibrosis].

[allergic bronchopulmonary aspergillosis]

The conditions for the prescription of inhaled steroids (ISs) in cystic fibrosis (CF) are not well established.To propose a formalized consensus agreement regarding the prescription of ISs in this disease.Application of the Delphi method in five thematic fields: indications, non-indications, dosage, precautions for use, and treatment follow-up.Thirty of forty-nine (61 %) reference CF centers in France participated in the process, which comprised three rounds. Experts strongly agreed that ISs are indicated in the presence of pulmonary manifestations with wheezing, personal history of atopy, and/or bronchial hyper-responsiveness. In contrast, ISs are not indicated as first-line therapy for allergic bronchopulmonary aspergillosis. Strong agreement was reached regarding the daily dose of ISs, which should be similar to what is given in asthma and adapted to control symptoms so as to prescribe the smallest possible dose. Increasing the frequency of bacterial and fungal sputum analyses and eye (cataract) assessments was not deemed necessary. However, in case of prolonged (>6months) use of high-dose ISs, monitoring bone mineral density and the hypothalamic-pituitary-adrenal axis, in particular if itraconazole is concomitantly prescribed, was recommended.This consensus statement defines a perimeter for the prescription of ISs in CF, with the aim of limiting their prescription (until new data are available).

Diseases presenting "bone mineral density" symptom

  • achondroplasia
  • adrenal incidentaloma
  • allergic bronchopulmonary aspergillosis
  • aromatase deficiency
  • congenital adrenal hyperplasia
  • cushing syndrome
  • dentinogenesis imperfecta
  • erythropoietic protoporphyria
  • fabry disease
  • familial hypocalciuric hypercalcemia
  • familial mediterranean fever
  • kallmann syndrome
  • lamellar ichthyosis
  • phenylketonuria
  • primary hyperoxaluria type 1

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