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Renal cystic disease of infancy: results of histochemical studies

A report of the Southwest Pediatric Nephrology Study Group

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Abstract

Histochemical techniques utilizingTetragonolobus lotus (proximal tubules),Arachis hypogaea (distal nephron, i.e., distal convoluted tubules and collecting ducts), and antibodies against Tamm-Horsfall protein (thick ascending limbs of Henle) were used to determine the site of origin of renal cysts in five children with autosomal recessive polycystic kidney disease (ARPKD) and three patients with glomerulocystic disease (GCD) presenting in the 1st year of life. The findings support a distal nephron origin for the cysts in the children who had ARPKD, whereas the majority of cysts in the children with GCD were confirmed as having a glomerular origin. Tamm-Horsfall protein was identified in the cysts of both ARPKD and GCD; this finding suggests free communication between some of the cysts with the thick ascending limb of Henle. An unexpected finding was the frequent presence of cysts surrounded by muscle fibers. We suggest that these cysts are of collecting duct origin.

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Southwest Pediatric Nephrology Study Group (SPNSG Central Office, Baylor University Medical Center at Dallas, Texas, USA): Director, Ronald J, Hogg: Associate Directors, Fred G, Administrative Assistant, Kaye Green. Participating Centers: Baylor College of Medicine, Houston, Texas, Phillip L. Berry, L. Leighton Hill, Sami A. Sanjad, Edith Hawkins; Baylor University Medical Center, Dallas, Texas, Ronald J. Hogg, Kaye Green; Tulane University Medical Center, New Orleans, Lonisiana, Frank Boineau, John E Lewy, Radhakrishna Baliga, Patrick Walker; University of Arkansas, Little Rock, Arkansas, Watson Arnold, Elleen Ellis, Edward Uthman; University of Colorado Health Science Center, Denver, Colorado, Gary M. Lum, William Hammond; University of Oklahoma Medical Center, Oklahoma City, Oklahoma, James Wenzl, James Matson. Geoffrey Altshuler, Sarah Johnson; University of Tennessee, Memphis, Tennessee, F. Bruder Stapleton, Shane Roy, III, Robert J. Wyatt, Charles McKay, William Murphy; University of Texas Health Science Center at Dallas, Texas, Billy S. Arant, Jr., Michel Baum, Fred G. Silva, Arthur Weinberg, Craig Argyle, Joseph Rutledge, Ed Eigenbrodt; University of Texas Medical School, Houston, Texas, Susan B. Conley, Jacques Lemire, Ron Portman, Ann Ince, Regina Verani; University of Texas Health Science Center at San Antonio, Texas, Michael Foulds, Sudesh Makker, Kanwal Kher, Melanie Sweet Victor Saldivar, Fermin Tio; University of Texas Medical Branch. Galveston, Texas, Ben H. Brouhard, Alok Kalia, Luther B Travis, Lisa Hollander, Tito Cavallo, Srinivasan Rajaramau; University of Utah Medical Center, Salt Lake City, Utah, Eileen Brewer, Richard Siegler, Elizabeth Hammond, Theodore Pysher; Washington University School of Medicine, St Louis, Missouri, Barbara R. Cole.

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Verani, R., Walker, P., Silva, F.G. et al. Renal cystic disease of infancy: results of histochemical studies. Pediatr Nephrol 3, 37–42 (1989). https://doi.org/10.1007/BF00859623

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  • DOI: https://doi.org/10.1007/BF00859623

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