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Hereditary Hematuria FREE

EDWIN P. RUSSELL, M.D.; NATHAN J. SMITH, M.D.
[+] Author Affiliations

Submitted for publication Jan. 19, 1959.


AMA Am J Dis Child. 1959;98(3):353-358. doi:10.1001/archpedi.1959.02070020355009.
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Idiopathic hematuria as a familial, genetically controlled disease is unusual. Reyersbach et al.1 identified this condition in only eight patients in an 18-year period in a large pediatric clinic. Hematuria, per se, is a commonly encountered clinical finding, and that this finding can result from some undefined genetically controlled abnormality must be frequently considered if rather disastrous diagnostic errors are to be avoided.

Three persons with asymptomatic hematuria and one who was asymptomatic for about 15 years and eventually succumbed to nephritis have been found in the family to be discussed here. An attempt has been made to gain some insight into the nature of the genetic transmission of the abnormality. The natural history of the disturbance is of interest with particular regard to the long-term prognosis in children found to have this condition.

Family History  The propositus, Case 1, was seen by one of us (E. P. R.)

REFERENCES

Reyersbach, G. C., and Butler, A. M.: Congenital Hereditary Hematuria, New England J. Med. 251:377-380, 1954;.
Goodwin, W. E.;  Alston E. F., and Semans, J. H.: Hematuria and Sickle Cell Disease , J. Urol. 63:79-96, 1950;.
Alport, A. C.:  Hereditary Familial Congenital Hemorrhagic Nephritis , Brit. M. J. 1:504-506, 1927;.
Guthrie, L. G.:  Idiopathic or Congenital Hereditary and Family Hematuria , Lancet 1:1243-1246, 1902;.
Wedgewood, R. J. P., and Klaus, M. H.:  Anaphylactoid Purpura: A Long Term Follow-Up Study with Special Reference to Renal Involvement , Pediatrics 16:196-207, 1955;.
Chapman, A. L.; Reeder, P. S.; Friedman, J. A., and Baker, L. A.:  Gross Hematuria in Sickle Cell Trait and Sickle Cell-Hemoglobin C Disease , Am. J. Med. 19:773-782, 1955;.
Reiman, H. A., and de Barardinis, C. T.:  Cyclic Disease , Medicine 30:219, 1951;.
Rinkoff, S. S.; Stern, A., and Schumer, H.:  Familial Nephritis , J. A. M. A. 113:661-664, 1939;.
Sturtz, G. S., and Burke, E. C.:  Syndrome of Hereditary Hematuria, Nephropathy, and Deafness , Proc. Staff Meet. Mayo Clin. 33:289-298, 1958;.

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References

Reyersbach, G. C., and Butler, A. M.: Congenital Hereditary Hematuria, New England J. Med. 251:377-380, 1954;.
Goodwin, W. E.;  Alston E. F., and Semans, J. H.: Hematuria and Sickle Cell Disease , J. Urol. 63:79-96, 1950;.
Alport, A. C.:  Hereditary Familial Congenital Hemorrhagic Nephritis , Brit. M. J. 1:504-506, 1927;.
Guthrie, L. G.:  Idiopathic or Congenital Hereditary and Family Hematuria , Lancet 1:1243-1246, 1902;.
Wedgewood, R. J. P., and Klaus, M. H.:  Anaphylactoid Purpura: A Long Term Follow-Up Study with Special Reference to Renal Involvement , Pediatrics 16:196-207, 1955;.
Chapman, A. L.; Reeder, P. S.; Friedman, J. A., and Baker, L. A.:  Gross Hematuria in Sickle Cell Trait and Sickle Cell-Hemoglobin C Disease , Am. J. Med. 19:773-782, 1955;.
Reiman, H. A., and de Barardinis, C. T.:  Cyclic Disease , Medicine 30:219, 1951;.
Rinkoff, S. S.; Stern, A., and Schumer, H.:  Familial Nephritis , J. A. M. A. 113:661-664, 1939;.
Sturtz, G. S., and Burke, E. C.:  Syndrome of Hereditary Hematuria, Nephropathy, and Deafness , Proc. Staff Meet. Mayo Clin. 33:289-298, 1958;.

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