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Proximal tubular cysts in fetal human autosomal recessive polycystic kidney disease.
J Am Soc Nephrol. 2000 Apr; 11(4):760-3.JA

Abstract

Standard texts describe human autosomal recessive polycystic kidney disease (ARPKD) as a cystic kidney disease in which lesions are localized to collecting tubules. Murine models of ARPKD consistently demonstrate an early phase of proximal tubular (PT) cystic involvement, which disappears shortly after birth. This is followed by a phase of collecting tubular (CT) cyst formation and progressive enlargement leading to compromise of renal function and death. Because the description of cystic lesions in human ARPKD has been largely based on postnatal specimens, PT cyst formation was hypothesized to be a characteristic feature of fetal human, as well as murine, ARPKD. This study examines nephron segment-specific cyst localization histochemically by lectin binding in 11 human ARPKD specimens obtained at different fetal and postnatal ages. PT cysts were found in human fetal specimens from gestational age 14 wk to 26 wk. The percentage of cysts involving PT segments ranged from 2 to 41%. The cystic index of PT cysts ranged from 2 to 5. In all specimens in which PT cysts were found, both the percentage of CT cysts and their cystic index were equal to or greater than the percentage of PT cysts and the associated PT cystic index. PT cysts were absent in all kidney specimens older than 34 wk gestational age. It is concluded that human ARPKD, like murine ARPKD, has a transient phase of PT cyst formation during early fetal development.

Authors+Show Affiliations

Department of Pediatrics, Rainbow Babies and Children's Hospital and Case Western Reserve University, Cleveland, Ohio, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

10752536

Citation

Nakanishi, K, et al. "Proximal Tubular Cysts in Fetal Human Autosomal Recessive Polycystic Kidney Disease." Journal of the American Society of Nephrology : JASN, vol. 11, no. 4, 2000, pp. 760-3.
Nakanishi K, Sweeney WE, Zerres K, et al. Proximal tubular cysts in fetal human autosomal recessive polycystic kidney disease. J Am Soc Nephrol. 2000;11(4):760-3.
Nakanishi, K., Sweeney, W. E., Zerres, K., Guay-Woodford, L. M., & Avner, E. D. (2000). Proximal tubular cysts in fetal human autosomal recessive polycystic kidney disease. Journal of the American Society of Nephrology : JASN, 11(4), 760-3.
Nakanishi K, et al. Proximal Tubular Cysts in Fetal Human Autosomal Recessive Polycystic Kidney Disease. J Am Soc Nephrol. 2000;11(4):760-3. PubMed PMID: 10752536.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Proximal tubular cysts in fetal human autosomal recessive polycystic kidney disease. AU - Nakanishi,K, AU - Sweeney,W E,Jr AU - Zerres,K, AU - Guay-Woodford,L M, AU - Avner,E D, PY - 2000/4/7/pubmed PY - 2000/6/10/medline PY - 2000/4/7/entrez SP - 760 EP - 3 JF - Journal of the American Society of Nephrology : JASN JO - J. Am. Soc. Nephrol. VL - 11 IS - 4 N2 - Standard texts describe human autosomal recessive polycystic kidney disease (ARPKD) as a cystic kidney disease in which lesions are localized to collecting tubules. Murine models of ARPKD consistently demonstrate an early phase of proximal tubular (PT) cystic involvement, which disappears shortly after birth. This is followed by a phase of collecting tubular (CT) cyst formation and progressive enlargement leading to compromise of renal function and death. Because the description of cystic lesions in human ARPKD has been largely based on postnatal specimens, PT cyst formation was hypothesized to be a characteristic feature of fetal human, as well as murine, ARPKD. This study examines nephron segment-specific cyst localization histochemically by lectin binding in 11 human ARPKD specimens obtained at different fetal and postnatal ages. PT cysts were found in human fetal specimens from gestational age 14 wk to 26 wk. The percentage of cysts involving PT segments ranged from 2 to 41%. The cystic index of PT cysts ranged from 2 to 5. In all specimens in which PT cysts were found, both the percentage of CT cysts and their cystic index were equal to or greater than the percentage of PT cysts and the associated PT cystic index. PT cysts were absent in all kidney specimens older than 34 wk gestational age. It is concluded that human ARPKD, like murine ARPKD, has a transient phase of PT cyst formation during early fetal development. SN - 1046-6673 UR - https://www.unboundmedicine.com/medline/citation/10752536/Proximal_tubular_cysts_in_fetal_human_autosomal_recessive_polycystic_kidney_disease_ L2 - http://jasn.asnjournals.org/cgi/pmidlookup?view=long&pmid=10752536 DB - PRIME DP - Unbound Medicine ER -