Glomerular lesions in congenital endocardial fibroelastosis. Clinical manifestations and ultrastructural studies in two patients

V. Pardo, R. Castro-Aballi, H. M. Goorman, J. Strauss

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Two children with congenital fibroelastosis and recurrent episodes of heart failure had overt proteinuria and hematuria; one also had a reversible nephrotic syndrome. Urinary manifestations persisted during periods of cardiac compensation. Renal biopsies revealed mesangial hyperplasia by light microscopy, identical ultrastructural lesions in the glomerular basement membrane, and deposits of fibrin in one of the biopsy specimens studied by immunofluorescence. These changes detected by electron microscopy may result in an increase in glomerular permeability independent of the renal hemodynamic disturbances associated with cardiac insufficiency. The progression of the lesions appears to be slow, although urinary manifestations may simulate an intercurrent glomerulonephritis. Pulmonary hypertension and renal venous stasis with glomerular intravascular coagulation were discussed as possible pathogenic mechanisms.

Original languageEnglish
Pages (from-to)410-413
Number of pages4
JournalArchives of Pathology and Laboratory Medicine
Volume102
Issue number8
StatePublished - Dec 1 1978

Fingerprint

Endocardial Fibroelastosis
Kidney
Biopsy
Glomerular Basement Membrane
Nephrotic Syndrome
Hematuria
Glomerulonephritis
Fibrin
Proteinuria
Pulmonary Hypertension
Hyperplasia
Fluorescent Antibody Technique
Microscopy
Permeability
Electron Microscopy
Heart Failure
Hemodynamics
Light

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Medical Laboratory Technology

Cite this

Glomerular lesions in congenital endocardial fibroelastosis. Clinical manifestations and ultrastructural studies in two patients. / Pardo, V.; Castro-Aballi, R.; Goorman, H. M.; Strauss, J.

In: Archives of Pathology and Laboratory Medicine, Vol. 102, No. 8, 01.12.1978, p. 410-413.

Research output: Contribution to journalArticle

@article{aff114a3462b43b2a201fef4af8359da,
title = "Glomerular lesions in congenital endocardial fibroelastosis. Clinical manifestations and ultrastructural studies in two patients",
abstract = "Two children with congenital fibroelastosis and recurrent episodes of heart failure had overt proteinuria and hematuria; one also had a reversible nephrotic syndrome. Urinary manifestations persisted during periods of cardiac compensation. Renal biopsies revealed mesangial hyperplasia by light microscopy, identical ultrastructural lesions in the glomerular basement membrane, and deposits of fibrin in one of the biopsy specimens studied by immunofluorescence. These changes detected by electron microscopy may result in an increase in glomerular permeability independent of the renal hemodynamic disturbances associated with cardiac insufficiency. The progression of the lesions appears to be slow, although urinary manifestations may simulate an intercurrent glomerulonephritis. Pulmonary hypertension and renal venous stasis with glomerular intravascular coagulation were discussed as possible pathogenic mechanisms.",
author = "V. Pardo and R. Castro-Aballi and Goorman, {H. M.} and J. Strauss",
year = "1978",
month = "12",
day = "1",
language = "English",
volume = "102",
pages = "410--413",
journal = "Archives of Pathology and Laboratory Medicine",
issn = "0003-9985",
publisher = "College of American Pathologists",
number = "8",

}

TY - JOUR

T1 - Glomerular lesions in congenital endocardial fibroelastosis. Clinical manifestations and ultrastructural studies in two patients

AU - Pardo, V.

AU - Castro-Aballi, R.

AU - Goorman, H. M.

AU - Strauss, J.

PY - 1978/12/1

Y1 - 1978/12/1

N2 - Two children with congenital fibroelastosis and recurrent episodes of heart failure had overt proteinuria and hematuria; one also had a reversible nephrotic syndrome. Urinary manifestations persisted during periods of cardiac compensation. Renal biopsies revealed mesangial hyperplasia by light microscopy, identical ultrastructural lesions in the glomerular basement membrane, and deposits of fibrin in one of the biopsy specimens studied by immunofluorescence. These changes detected by electron microscopy may result in an increase in glomerular permeability independent of the renal hemodynamic disturbances associated with cardiac insufficiency. The progression of the lesions appears to be slow, although urinary manifestations may simulate an intercurrent glomerulonephritis. Pulmonary hypertension and renal venous stasis with glomerular intravascular coagulation were discussed as possible pathogenic mechanisms.

AB - Two children with congenital fibroelastosis and recurrent episodes of heart failure had overt proteinuria and hematuria; one also had a reversible nephrotic syndrome. Urinary manifestations persisted during periods of cardiac compensation. Renal biopsies revealed mesangial hyperplasia by light microscopy, identical ultrastructural lesions in the glomerular basement membrane, and deposits of fibrin in one of the biopsy specimens studied by immunofluorescence. These changes detected by electron microscopy may result in an increase in glomerular permeability independent of the renal hemodynamic disturbances associated with cardiac insufficiency. The progression of the lesions appears to be slow, although urinary manifestations may simulate an intercurrent glomerulonephritis. Pulmonary hypertension and renal venous stasis with glomerular intravascular coagulation were discussed as possible pathogenic mechanisms.

UR - http://www.scopus.com/inward/record.url?scp=0018135051&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0018135051&partnerID=8YFLogxK

M3 - Article

VL - 102

SP - 410

EP - 413

JO - Archives of Pathology and Laboratory Medicine

JF - Archives of Pathology and Laboratory Medicine

SN - 0003-9985

IS - 8

ER -