TY - JOUR AU - Miller-Hjelle, Marcia A. AU - Hjelle, J. Thomas AU - Jones, Monica AU - Mayberry, William R. AU - Dombrink-Kurtzman, Mary Ann AU - Peterson, Stephen W. AU - Nowak, Deborah M. AU - Darras, Frank S. T1 - Polycystic Kidney Disease: An Unrecognized Emerging Infectious Disease? T2 - Emerging Infectious Disease journal PY - 1997 VL - 3 IS - 2 SP - 113 SN - 1080-6059 AB - Polycystic kidney disease (PKD) is one of the most common genetic diseases in humans. We contend that it may be an emerging infectious disease and/or microbial toxicosis in a vulnerable human subpopulation. Use of a differential activation protocol for the Limulus amebocyte lysate (LAL) assay showed bacterial endotoxin and fungal (13)-ß-D-glucans in cyst fluids from human kidneys with PKD. Fatty acid analysis of cyst fluid confirmed the presence of 3-hydroxy fatty acids characteristic of endotoxin. Tissue and cyst fluid from three PKD patients were examined for fungal components. Serologic tests showed Fusarium, Aspergillus, and Candida antigens. IgE, but not IgG, reactive with Fusarium and Candida were also detected in cyst fluid. Fungal DNA was detected in kidney tissue and cyst fluid from these three PKD patients, but not in healthy human kidney tissue. We examine the intertwined nature of the actions of endotoxin and fungal components, sphingolipid biology in PKD, the structure of PKD gene products, infections, and integrity of gut function to establish a mechanistic hypothesis for microbial provocation of human cystic disease. Proof of this hypothesis will require identification of the microbes and microbial components involved and multifaceted studies of PKD cell biology. KW - United States DO - 10.3201/eid0302.970204 UR - https://wwwnc.cdc.gov/eid/article/3/2/97-0204_article ER - End of Reference