title: Diagnostic Algorithm in the Management of Acute Febrile Abdomen in Patients with Autosomal Dominant Polycystic Kidney Disease.
abstract: BACKGROUND
  Acute febrile abdomen represents a diagnostic challenge in patients with autosomal dominant polycystic kidney disease (ADPKD). Although criteria have been proposed for cyst infection (CyI) and hemorrhage (CyH), there is a lack of comparative assessments. Furthermore, distinguishing cystic from non-cystic complications remains problematic.
  
  
  METHODS
  ADPKD patients presenting with abdominal pain and/or fever between 01/2005 and 06/2015 were retrospectively identified in a systematic computerized billing database. CyH was defined as spontaneous intracystic density above 50 Hounsfield units on computed tomography (CT). CyI was definite if confirmed by cyst puncture, and probable if 4 criteria were met: 3-day fever, loin/liver tenderness, C-reactive protein (CRP) plasma levels >50mg/L and no CT evidence for CyH. Other episodes were grouped as inflammation of unknown origin (IUO).
  
  
  RESULTS
  Among a cohort of 173 ADPKD patients, 101 presented with 205 episodes of abdominal pain (n = 172) and/or fever (n = 33). 20 patients experienced 30 CyH, whereas 16 presented 23 episodes of definite (n = 11) or probable (n = 12) CyI. 35 IUO were observed in 31 patients. Clinically, fever was observed in 7% vs. 100% vs. 66% of CyH, CyI and IUO, respectively. Biologically, CRP cut-off at 70 mg/dl showed 92% sensitivity and 81% specificity in CyI diagnosis. Urine or blood cultures remained sterile in >90% of CyH, but were contributive in 53.4% of CyI and IUO, with a 74.2% prevalence for E. coli. Radiologically, ultrasounds, CT and magnetic resonance diagnosed CyI in 2.6%, 20% and 16.7% of cases, respectively. 18F-FDG positron-emission tomography (PET)/CT was done within a median period of 7 days post antibiotics, and significantly changed patient management in 71.4%.
  
  
  CONCLUSIONS
  This retrospective single-center series underscores the usefulness of clinical-fever-and biological-CRP-parameters, but emphasizes the limitations of bacteriological and radiological investigations in cases of acute febrile abdomen in ADPKD patients. 18F-FDG-PET/CT imaging may be helpful in such condition.
authors.affiliation: Department of Nephrology, University of Liège Hospital (ULg CHU), Liège, Belgium.
authors.forename: Marie
authors.initials: M
authors.lastname: Neuville
authors.affiliation: Department of Nuclear Medicine, University of Liège Hospital (ULg CHU), Liège, Belgium.
authors.forename: Roland
authors.initials: R
authors.lastname: Hustinx
authors.affiliation: Department of Medico-Economic Information, University of Liège Hospital (ULg CHU), Liège, Belgium.
authors.forename: Jessica
authors.initials: J
authors.lastname: Jacques
authors.affiliation: Department of Nephrology, University of Liège Hospital (ULg CHU), Liège, Belgium.|Groupe Interdisciplinaire de Génoprotéomique Appliquée (GIGA), Cardiovascular Sciences, University of Liège, Liège, Belgium.
authors.forename: Jean-Marie
authors.initials: JM
authors.lastname: Krzesinski
authors.affiliation: Department of Nephrology, University of Liège Hospital (ULg CHU), Liège, Belgium.|Groupe Interdisciplinaire de Génoprotéomique Appliquée (GIGA), Cardiovascular Sciences, University of Liège, Liège, Belgium.
authors.forename: François
authors.identifier: http://orcid.org/0000-0003-2547-6593
authors.initials: F
authors.lastname: Jouret
mesh_terms: D015746:Abdominal Pain / Q000150:complications*; D000208:Acute Disease; D000368:Aged; D000465:Algorithms; D015331:Cohort Studies; D003560:Cysts / Q000150:complications; D005260:Female; D005334:Fever / Q000150:complications*; D019788:Fluorodeoxyglucose F18; D006801:Humans; D008297:Male; D008875:Middle Aged; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000000981:diagnostic imaging*; D000072078:Positron Emission Tomography Computed Tomography; D012189:Retrospective Studies
chemical_list: D019788:Fluorodeoxyglucose F18