Rare Diseases Symptoms Automatic Extraction

Incidence of Pneumocystis jiroveci Pneumonia among Groups at Risk in HIV-negative Patients.

[waldenström macroglobulinemia]

Pneumocystis jiroveci pneumonia in HIV-negative immunocompromised patients is associated with high mortality rates. Although trimethoprim-sulfamethoxazole (TMP-SMX) provides a very effective prophylaxis, pneumocystosis still occurs and may even be emerging, due to sub-optimal characterization of patients most at risk, hence precluding targeted prophylaxis.We retrospectively analyzed all cases of documented pneumocystosis in HIV-negative patients admitted in our institution, a referral center in the area, from January 1990 to June 2010, and extracted data on their underlying condition(s). To estimate incidence rates within each condition, we estimated the number of patients followed-up in our area for each condition, by measuring the number of patients admitted with the corresponding international classification diagnostic code, through the national hospital discharge database (PMSI).From 1990 to 2010, 293 cases of pneumocystosis were documented, of whom 154 (52.6%) tested negative for HIV. The main underlying conditions were hematological malignancies (32.5%), solid tumors (18.2%), inflammatory diseases (14.9%), solid organ transplant (12.3%), and vasculitis (9.7%). Estimated incidence rates could be ranked in three categories: i) high risk (incidence rates > 45 cases per 100,000 patient-year): polyarteritis nodosa, granulomatosis with polyangiitis, polymyositis/dermatopolymyositis, acute leukemia, chronic lymphocytic leukemia, and non-Hodgkin lymphoma; ii) intermediate risk (25-45 cases per 100,000 patient-year): Waldenström macroglobulinemia, multiple myeloma, and central nervous system cancer; and iii) low risk (< 25 cases per 100,000 patient-year): other solid tumors, inflammatory diseases, and Hodgkin lymphoma.These estimates may be used as a guide to better target pneumocystosis prophylaxis in the groups most at risk.

Diseases presenting "low risk" symptom

  • achondroplasia
  • congenital diaphragmatic hernia
  • esophageal adenocarcinoma
  • heparin-induced thrombocytopenia
  • hodgkin lymphoma, classical
  • locked-in syndrome
  • primary hyperoxaluria type 1
  • sneddon syndrome
  • waldenström macroglobulinemia

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