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Focal Glomerular Sclerosis: Clinical, Laboratory, Pathological Findings, Treatment and Prognosis (20 Cases)

Year 1978, Volume: 31 Issue: 3, 672 - 680, 30.09.1978

Necmiye Tümer

Abstract

Bu çalışmada, fokal glomerüler skleroz tanısı konmuş 20 çocuk hastanın klinik, laboratuvar ve patolojik bulguları, uygulanan tedaviler ve hastalığın prognozu değerlendirilmiştir. Vakaların çoğunda ödem, proteinüri, mikroskopik hematüri ve steroid tedavisine direnç gözlenmiştir. Hastalığın ilerleyen dönemlerinde böbrek fonksiyonlarında bozulma ve bazı vakalarda hemodiyaliz gereksinimi ortaya çıkmıştır. Ayrıca, başlangıçta hipertansiyon ve hematüri bulunan hastalarda böbrek fonksiyonlarının daha erken bozulduğu saptanmıştır. Literatür bulguları ışığında, fokal glomerüler sklerozun kötü prognozlu bir antite olduğu ve erken tanı ile ayırıcı tanısının önem taşıdığı vurgulanmıştır.

Keywords

Fokal glomerüler skleroz nefrotik sendrom proteinüri mikroskopik hematüri böbrek yetmezliği steroid direnci çocuk nefrolojisi

Project Number

-

References

  • Arneil, G.C. : The Nephrotic Syndrome. Pediatr. Clin. N. Am. 18 : 547, 1971.
  • Arneil, G.C. : Treatment of the Nephrosis with Predisolone. Lancet. 4 : 409 1956.
  • Barratt, T.M., Osofsky, S.G., Bercowsky, As, Soothill, J.F. : Cyclophosphamide Treatment in steroid - Sensitive Nephrotic Syndrome of Childhood. Lancet. 1 • 55, 1975.
  • Berger, J. Yaneva, H., Hinglais, H., Hinglais, N. : Immunohistochemistry of Flomerulonephritis. Year Book Med. Publ. 11, 1971
  • Churg, J., Habib, R., White, R.H.R. : Pathology of the Nephrotic Syndrome in Children. Lancet. 6 : 20, 1970.
  • Cornfeld, D, Schwarts, M.W. : Nephrosis. A Long-Term Study of Children Treated with Corticosteroids. J. Pediatr. 68 • 507, 1966.
  • Glasgow, E.F. White, R.H.R. : «Not so Minimal». Nephron. 8 : 403, 1971.
  • Habib, R. : Focal Glomerular Sclerosis. Kidney Int. 4 : 355, 1973.
  • Habib, R., Kleinknecht, C. : The Primary Nephrotic Syndrome of Childlhood. Classification and Clinicopathologic Study of 406 Cases. Path. Ann. New York. : 417, 1971.
  • Hayslett, J.P., Krassner, L.S., Bensch, K.G., Kashgarian, M., Epstein, F.H. : Progression of «Lipoid Nephrosis» to Renal Insufficiency. New Engl. J. Med. 281 181, 1969.
  • Hoyer, J.R., Vernier, R.L., Najarian, J.S., Raij, L, Simmons, R.L., Michael, A.F. Recurrence of Idiopathic Nephrotic Syndrome After Renal Transplantation, Lancet. 8 19, 1972.
  • Hyman, L.R., Burkholder, P.M. : Focal Sclerosing Glomerulonephropathy with Hya linosis. J..84 : 217, 1974
  • Hyman, R.L. Burkholder, P.M. : Focal Sclerosing Glomerulonephropathy with Segmental Hyalinosis. Lab. Invest. 28 : 533, 1973.
  • Jenis, E.H., Teichman, S.T., Briggs, W.A., Sandler, P., Hollerman, C.E. Calcagno, Tižmer
  • P.L., Knieser, MR., Jensen, G.E., Valeski, J.E. : Focal Segmental Glomerulosclerosis, Am J. Med. 57 : 695, 1974.
  • Kaplan, B.S., Bureau, M.A., Drummond, KN. : The Nephrotic Syndrome in the First Year of Life. Is a Pathologic Classification Possible? J. Pediatr. 85 : 615 1974.
  • Kashgarian, M., Hayslett, J.P., Siegel, N.J. : Lipoid Nephrosis and Focal Sclerosis Distinct Entities or Spectrum of Disease. Nephron. 13 105, 1974.
  • Makker, S.P., Heymann, W. : The Idiopathic Nephrotic Syndrome of Childhood. Am. J. Dis. Child. 127 : 830, 1974.
  • McDonald, R., Wiggelinkhuizen, J., Kashula, R.O.C. : The Nephrotic Syndrome in very Young Infants. Am. J. Dis. Child. 122 : 507, 1971.
  • McGovern, V.J. : Persistent Nephrotic Syndrome. A Renal Biopsy Study. Australasian Ann. Med. 13 : 306, 1964.
  • Michael, A.F. : The Nephrotic Eyndrome. Phase B. Kidney and Urinary Tract. 87, 1973.
  • Michael, A.F., Fish, A. : Immunological Mechanism in Renal Disease. Phase B. Kidney and Urinary Tract. : 53, 1973.
  • Nagi, A.H., Alexander, F., Lannigan, R. : Light and Electron Microscopical Studies of Focal Glomerular Sclerosis, J. Clin. Path. 24 : 846, 1971.
  • Pennisi, A.J., Grushkin, C.M., Lieberman, E. : Gonodal Function in Children with Nephrosis Treated with Cyclophosphamide. Am. J. Dis. Child. 129 : 315, 1975.
  • Raij, L, Hoyer, R.L., Michael, A.F. : Steroid-Resistant Nephrotic Syndrome. Recurrence After Transplantation. Ann. Intern. Med. 77 : 581, 1972.
  • Rich, A.R. : A Hitherto Undescribed Vulnerability of the Juxtamedullary Glomeruli in Lipoid Nehprosis. Bull. John Hopkins Hosp. 100 : 173, 1957.
  • Royer, P., Habib, R., Mathieu H., Broyer, M. : Pediatric Nephrology. W.B. Saunders co., Phila., London, 1974.
  • Saxena, K.M., Grawford, J.D. : Current Concepts. The Treatment of Nephrosis. New Engl. J. Med. 272 : 522, 1965.
  • Siegel, N.J., Kashgarian, M., Spargo, B.H., Hayslett, J.P. : Minimal Change and Focal Sclerotic Lesions in Lipoid Nephrosis. Nephron. 13 : 125, 1974.
  • Siegel, N.J., Goldberg, B., Krasner, L.S., Hayslett, J.P. : Long-Term Follow-Up of Child ren with Steroid-Rcsponsive Nephrotic Syndrome. J. Pediatr. 81 : 251, 1972.
  • Smith, F.G., Gonick, H., Stanley, T.M., McIntosh, R.M. : The Nephrotic Syndrome. Current Copcepts. Ann. Intern. Med. 76 : 563, 1972.
  • Strauss, M.B., Welt, LG. : Diseases of the Kidney. Sec. Edit. Little, Brown and Co., Boston, 1971.
  • White, R.H.R., Glavkow, E.F., Mills, R.J. : Clinicopathological study of Nephrotic Syndrome in Chlidhood. Lancet. 6 : 1353, 1970.
  • Vaughan, V.C., McKay, R.J., Nelson, w.E. : Textbook of Pediatrics. w.B. Saunders co. Phila. and London, Toronto, 1975.

Fokal Glomerüler Skleroz:Klinik, Laboratuvar,Patolojik Bulgular, Tedavi Ve Prognozu(20 Olgu Nedeniyle)

Year 1978, Volume: 31 Issue: 3, 672 - 680, 30.09.1978

Necmiye Tümer

Abstract

This study evaluates the clinical, laboratory, and pathological findings, treatments, and prognosis of 20 pediatric patients diagnosed with focal glomerular sclerosis. Most cases presented with edema, proteinuria, microscopic hematuria, and resistance to steroid therapy. Progressive deterioration in renal function and the need for hemodialysis were observed in several patients. Notably, earlier renal impairment was found in patients who initially presented with hypertension and hematuria. In light of the literature, focal glomerular sclerosis is emphasized as a poor-prognosis entity, underlining the importance of early diagnosis and differential assessment.

Keywords

Focal glomerular sclerosis nephrotic syndrome proteinuria microscopic hematuria renal failure steroid resistance pediatric nephrology

Ethical Statement

-

Supporting Institution

-

Project Number

-

Thanks

-

References

  • Arneil, G.C. : The Nephrotic Syndrome. Pediatr. Clin. N. Am. 18 : 547, 1971.
  • Arneil, G.C. : Treatment of the Nephrosis with Predisolone. Lancet. 4 : 409 1956.
  • Barratt, T.M., Osofsky, S.G., Bercowsky, As, Soothill, J.F. : Cyclophosphamide Treatment in steroid - Sensitive Nephrotic Syndrome of Childhood. Lancet. 1 • 55, 1975.
  • Berger, J. Yaneva, H., Hinglais, H., Hinglais, N. : Immunohistochemistry of Flomerulonephritis. Year Book Med. Publ. 11, 1971
  • Churg, J., Habib, R., White, R.H.R. : Pathology of the Nephrotic Syndrome in Children. Lancet. 6 : 20, 1970.
  • Cornfeld, D, Schwarts, M.W. : Nephrosis. A Long-Term Study of Children Treated with Corticosteroids. J. Pediatr. 68 • 507, 1966.
  • Glasgow, E.F. White, R.H.R. : «Not so Minimal». Nephron. 8 : 403, 1971.
  • Habib, R. : Focal Glomerular Sclerosis. Kidney Int. 4 : 355, 1973.
  • Habib, R., Kleinknecht, C. : The Primary Nephrotic Syndrome of Childlhood. Classification and Clinicopathologic Study of 406 Cases. Path. Ann. New York. : 417, 1971.
  • Hayslett, J.P., Krassner, L.S., Bensch, K.G., Kashgarian, M., Epstein, F.H. : Progression of «Lipoid Nephrosis» to Renal Insufficiency. New Engl. J. Med. 281 181, 1969.
  • Hoyer, J.R., Vernier, R.L., Najarian, J.S., Raij, L, Simmons, R.L., Michael, A.F. Recurrence of Idiopathic Nephrotic Syndrome After Renal Transplantation, Lancet. 8 19, 1972.
  • Hyman, L.R., Burkholder, P.M. : Focal Sclerosing Glomerulonephropathy with Hya linosis. J..84 : 217, 1974
  • Hyman, R.L. Burkholder, P.M. : Focal Sclerosing Glomerulonephropathy with Segmental Hyalinosis. Lab. Invest. 28 : 533, 1973.
  • Jenis, E.H., Teichman, S.T., Briggs, W.A., Sandler, P., Hollerman, C.E. Calcagno, Tižmer
  • P.L., Knieser, MR., Jensen, G.E., Valeski, J.E. : Focal Segmental Glomerulosclerosis, Am J. Med. 57 : 695, 1974.
  • Kaplan, B.S., Bureau, M.A., Drummond, KN. : The Nephrotic Syndrome in the First Year of Life. Is a Pathologic Classification Possible? J. Pediatr. 85 : 615 1974.
  • Kashgarian, M., Hayslett, J.P., Siegel, N.J. : Lipoid Nephrosis and Focal Sclerosis Distinct Entities or Spectrum of Disease. Nephron. 13 105, 1974.
  • Makker, S.P., Heymann, W. : The Idiopathic Nephrotic Syndrome of Childhood. Am. J. Dis. Child. 127 : 830, 1974.
  • McDonald, R., Wiggelinkhuizen, J., Kashula, R.O.C. : The Nephrotic Syndrome in very Young Infants. Am. J. Dis. Child. 122 : 507, 1971.
  • McGovern, V.J. : Persistent Nephrotic Syndrome. A Renal Biopsy Study. Australasian Ann. Med. 13 : 306, 1964.
  • Michael, A.F. : The Nephrotic Eyndrome. Phase B. Kidney and Urinary Tract. 87, 1973.
  • Michael, A.F., Fish, A. : Immunological Mechanism in Renal Disease. Phase B. Kidney and Urinary Tract. : 53, 1973.
  • Nagi, A.H., Alexander, F., Lannigan, R. : Light and Electron Microscopical Studies of Focal Glomerular Sclerosis, J. Clin. Path. 24 : 846, 1971.
  • Pennisi, A.J., Grushkin, C.M., Lieberman, E. : Gonodal Function in Children with Nephrosis Treated with Cyclophosphamide. Am. J. Dis. Child. 129 : 315, 1975.
  • Raij, L, Hoyer, R.L., Michael, A.F. : Steroid-Resistant Nephrotic Syndrome. Recurrence After Transplantation. Ann. Intern. Med. 77 : 581, 1972.
  • Rich, A.R. : A Hitherto Undescribed Vulnerability of the Juxtamedullary Glomeruli in Lipoid Nehprosis. Bull. John Hopkins Hosp. 100 : 173, 1957.
  • Royer, P., Habib, R., Mathieu H., Broyer, M. : Pediatric Nephrology. W.B. Saunders co., Phila., London, 1974.
  • Saxena, K.M., Grawford, J.D. : Current Concepts. The Treatment of Nephrosis. New Engl. J. Med. 272 : 522, 1965.
  • Siegel, N.J., Kashgarian, M., Spargo, B.H., Hayslett, J.P. : Minimal Change and Focal Sclerotic Lesions in Lipoid Nephrosis. Nephron. 13 : 125, 1974.
  • Siegel, N.J., Goldberg, B., Krasner, L.S., Hayslett, J.P. : Long-Term Follow-Up of Child ren with Steroid-Rcsponsive Nephrotic Syndrome. J. Pediatr. 81 : 251, 1972.
  • Smith, F.G., Gonick, H., Stanley, T.M., McIntosh, R.M. : The Nephrotic Syndrome. Current Copcepts. Ann. Intern. Med. 76 : 563, 1972.
  • Strauss, M.B., Welt, LG. : Diseases of the Kidney. Sec. Edit. Little, Brown and Co., Boston, 1971.
  • White, R.H.R., Glavkow, E.F., Mills, R.J. : Clinicopathological study of Nephrotic Syndrome in Chlidhood. Lancet. 6 : 1353, 1970.
  • Vaughan, V.C., McKay, R.J., Nelson, w.E. : Textbook of Pediatrics. w.B. Saunders co. Phila. and London, Toronto, 1975.
There are 34 citations in total.

Details

Primary Language English
Subjects Pediatric Nephrology
Journal Section Articles
Authors

Necmiye Tümer This is me

Project Number -
Publication Date September 30, 1978
Published in Issue Year 1978 Volume: 31 Issue: 3

Cite

APA Tümer, N. (1978). Fokal Glomerüler Skleroz:Klinik, Laboratuvar,Patolojik Bulgular, Tedavi Ve Prognozu(20 Olgu Nedeniyle). Ankara Üniversitesi Tıp Fakültesi Mecmuası, 31(3), 672-680.
AMA Tümer N. Fokal Glomerüler Skleroz:Klinik, Laboratuvar,Patolojik Bulgular, Tedavi Ve Prognozu(20 Olgu Nedeniyle). Ankara Üniversitesi Tıp Fakültesi Mecmuası. September 1978;31(3):672-680.
Chicago Tümer, Necmiye. “Fokal Glomerüler Skleroz:Klinik, Laboratuvar,Patolojik Bulgular, Tedavi Ve Prognozu(20 Olgu Nedeniyle)”. Ankara Üniversitesi Tıp Fakültesi Mecmuası 31, no. 3 (September 1978): 672-80.
EndNote Tümer N (September 1, 1978) Fokal Glomerüler Skleroz:Klinik, Laboratuvar,Patolojik Bulgular, Tedavi Ve Prognozu(20 Olgu Nedeniyle). Ankara Üniversitesi Tıp Fakültesi Mecmuası 31 3 672–680.
IEEE N. Tümer, “Fokal Glomerüler Skleroz:Klinik, Laboratuvar,Patolojik Bulgular, Tedavi Ve Prognozu(20 Olgu Nedeniyle)”, Ankara Üniversitesi Tıp Fakültesi Mecmuası, vol. 31, no. 3, pp. 672–680, 1978.
ISNAD Tümer, Necmiye. “Fokal Glomerüler Skleroz:Klinik, Laboratuvar,Patolojik Bulgular, Tedavi Ve Prognozu(20 Olgu Nedeniyle)”. Ankara Üniversitesi Tıp Fakültesi Mecmuası 31/3 (September1978), 672-680.
JAMA Tümer N. Fokal Glomerüler Skleroz:Klinik, Laboratuvar,Patolojik Bulgular, Tedavi Ve Prognozu(20 Olgu Nedeniyle). Ankara Üniversitesi Tıp Fakültesi Mecmuası. 1978;31:672–680.
MLA Tümer, Necmiye. “Fokal Glomerüler Skleroz:Klinik, Laboratuvar,Patolojik Bulgular, Tedavi Ve Prognozu(20 Olgu Nedeniyle)”. Ankara Üniversitesi Tıp Fakültesi Mecmuası, vol. 31, no. 3, 1978, pp. 672-80.
Vancouver Tümer N. Fokal Glomerüler Skleroz:Klinik, Laboratuvar,Patolojik Bulgular, Tedavi Ve Prognozu(20 Olgu Nedeniyle). Ankara Üniversitesi Tıp Fakültesi Mecmuası. 1978;31(3):672-80.