title: Zygomycosis in children: a systematic review and analysis of reported cases.
abstract: BACKGROUND
  Zygomycosis has emerged as an increasingly important infection with a high mortality especially in immunocompromised patients. No comprehensive analysis of pediatric zygomycosis cases has been published to date.
  
  
  METHODS
  We used a PUBMED search for English publications of pediatric (0-18 years) zygomycosis cases and references from major books as well as single case reports or case series. Individual references were reviewed for additional cases. Data were entered into Filemaker-pro database and analyzed by logistic regression analysis.
  
  
  RESULTS
  One hundred fifty-seven cases (64% male) were found with median age 5 years (range, 0.16-13). Underlying conditions included neutropenia (18%), prematurity (17%), diabetes mellitus (15%), ketoacidosis (10%), and no apparent underlying condition (14%). The most common patterns of zygomycosis were cutaneous (27%), gastrointestinal (21%), rhinocerebral (18%), and pulmonary (16%). Among 77 culture-confirmed cases, Rhizopus spp. (44%) and Mucor spp. (15%) were most commonly identified. Of 81 patients who were given antifungal therapy, 73% received an amphotericin B formulation only. The remaining patients received mostly amphotericin B in combination with other antifungal agents. Mortality in patients without antifungal therapy was higher than in those with therapy (88% versus 36%, P < 0.0001). Ninety-two (59%) patients underwent surgery. Cerebral, gastrointestinal, disseminated and cutaneous zygomycosis were associated with mortality rates of 100, 100, 88, and 0%, respectively. Independent risk factors for death were disseminated infection (OR: 7.18; 95% CI: 3.02-36.59) and age <1 year (OR: 3.85; 95% CI: 1.05-7.43). Antifungal therapy and particularly surgery reduced risk of death by 92% (OR: 0.07; 95% CI: 0.04-0.25) and 84% (OR: 0.16; 95% CI: 0.09-0.61), respectively.
  
  
  CONCLUSIONS
  Zygomycosis is a life-threatening infection in children with neutropenia, diabetes mellitus, and prematurity as common predisposing factors, and there is high mortality in untreated disease, disseminated infection, and age <1 year. Amphotericin B and surgery significantly improve outcome.
authors.affiliation: Department of Pediatrics and the Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania School of Medicine, Philadelphia, PA, USA. zaoutis@email.chop.edu
authors.forename: Theoklis E
authors.initials: TE
authors.lastname: Zaoutis
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Roilides
authors.forename: Christine C
authors.initials: CC
authors.lastname: Chiou
authors.forename: Wendy L
authors.initials: WL
authors.lastname: Buchanan
authors.forename: Tena A
authors.initials: TA
authors.lastname: Knudsen
authors.forename: Tatyana A
authors.initials: TA
authors.lastname: Sarkisova
authors.forename: Robert L
authors.initials: RL
authors.lastname: Schaufele
authors.forename: Michael
authors.initials: M
authors.lastname: Sein
authors.forename: Tin
authors.initials: T
authors.lastname: Sein
authors.forename: Priya A
authors.initials: PA
authors.lastname: Prasad
authors.forename: Jaclyn H
authors.initials: JH
authors.lastname: Chu
authors.forename: Thomas J
authors.initials: TJ
authors.lastname: Walsh
mesh_terms: D000293:Adolescent; D000935:Antifungal Agents / Q000627:therapeutic use; D020314:Central Nervous System Fungal Infections / Q000382:microbiology; D002648:Child; D002675:Child, Preschool; D003881:Dermatomycoses / Q000382:microbiology; D048909:Diabetes Complications; D004359:Drug Therapy, Combination; D005260:Female; D005767:Gastrointestinal Diseases / Q000382:microbiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007234:Infant, Premature; D008171:Lung Diseases / Q000382:microbiology; D008297:Male; D009503:Neutropenia / Q000150:complications; D012307:Risk Factors; D016896:Treatment Outcome; D020096:Zygomycosis / Q000188:drug therapy / Q000453:epidemiology* / Q000382:microbiology* / Q000503:physiopathology
chemical_list: D000935:Antifungal Agents