Düşük Kompleman Düzeyi Olan Henoch Schönlein Purpuralı Çocuk Hastaların Değerlendirilmesi

Özge Başaran

doi:10.12956/tjpd.2018.367

Research Article

Düşük Kompleman Düzeyi Olan Henoch Schönlein Purpuralı Çocuk Hastaların Değerlendirilmesi

Year 2019, Volume: 13 Issue: 3, 208 - 212, 24.05.2019

Özge Başaran

https://doi.org/10.12956/tjpd.2018.367

Abstract

Amaç: Henoch Schönlein purpurası (HSP) çocukluk çağında en sık rastlanan sistemik vaskülittir. Her ne kadar HSP’nin

patogenezinde kompleman sisteminin rol oynadığı düşünülmekteyse de düşük kompleman 3 (C3) ve kompleman 4

(C4) düzeyleri beklenen bir bulgu değildir. Çalışma ile hastanemiz çocuk romatoloji kliniğinde takip edilmekte olan ve

kompleman düzeylerinde düşüklük saptanan HSP hastaların demografik ve klinik özelliklerinin geriye dönük olarak değerlendirilmesi

yapılmıştır.

Gereç ve Yöntemler: Ocak 2010 - Aralık 2017 tarihleri arasında hastanemiz çocuk romatoloji kliniğinde izlenen 759

HSP hastasının dosyaları geriye dönük olarak tarandı. C3 ve C4 düzeyi düşük saptanan 17 hasta çalışmaya dâhil edildi.

Hastaların demografik ve klinik özellikleri ile laboratuvar sonuçları değerlendirildi.

Bulgular: Hastaların başvuru anındaki yaş ortalaması 8.93 (8-14) yıldı. Hastaların toplam izlem süre ortalaması 24.4

(3-84) aydı. On bir hasta erkek, 6 hasta ise kızdı. Hastaların hepsinde cilt tutulumu vardı. Yedi hastada gastrointestinal

sistem tutulumu, 12 hastada eklem tutulumu, 1 hastada testis tutulumu, 5 hastada renal tutulum mevcuttu. Üç hastanın

izlem süreleri boyunca kompleman seviyeleri normal sınırlara çıkmadı. Kalan hastalarda kompleman seviyesi normal

sınırlara yükseldi.

Sonuç: Kompleman seviyelerinde düşüklük HSP hastalarında beklenebilecek bir bulgudur. Büyük çoğunluğu geçici

olan bu düşüklüğün hastalığın seyri ve klinik bulguları ile ilişkisi saptanmamıştır. Ancak bu hastaların ileri dönem ortaya

çıkabilecek otoimmün hastalıklar (SLE vb.) açısından daha uzun süreli izlemlerine ihtiyaç vardır.

Keywords

Çocuk, Henoch Schönlein purpurası

References

1. McCarthy HJ, Tizard EJ. Clinical practice: Diagnosis and management of Henoch-Schönlein purpura. Eur J Pediatr 2010;169:643-50.
2. Ballinger S. Henoch-Schonlein purpura. Curr Opin Rheumatol 2003;15:591-4.
3. Lau KK, Suzuki H, Novak J, Wyatt RJ. Pathogenesis of Henoch- Schönlein purpura nephritis. Pediatr Nephrol 2010;25:19-26.
4. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013;65:1-11.
5. Hisano S, Matsushita M, Fujita T, Takeshita M, Iwasaki H. Activation of the lectin complement pathway in post-streptococcal acute glomerulonephritis. Pathol Int 2007;57:351-7.
6. Garcia-Fuentes M, Martin A, Chantler C, Williams DG. Serum complement components in Henoch-Schönlein purpura. Arch Dis Child 1978;53:417-9.
7. Islek I, Muslu A, Dagdemir A, Dilber C. Is low serum complement 3 in Henoch Schönlein purpura unusual and of prognostic value? Eur J Pediatr 2001;160:397-8.
8. Motoyama O, Iitaka K. Henoch-Schonlein purpura with hypocomplementemia in children. Pediatr Int 2005;47:39-42.
9. Lin Q, Min Y, Li Y, Zhu Y, Song X, Xu Q, et al. Henoch-Schönlein purpura with hypocomplementemia. Pediatr Nephrol 2012;27:801- 6.
10. Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. Paediatric Rheumatology International Trials Organisation (PRINTO). EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 2010;69:798- 806.
11. Krause I, Garty BZ, Davidovits M, Cleper R, Tamary H, Rosenmann E, et al. Low serum C3, leukopenia, and thrombocytopenia: Unusual features of henoch-schonlein purpura. Eur J Pediatr 1999;158:906-9.
12. Levy M, Broyer M, Arsan A, Levy-Bentolila D, Habib R. Anaphylactoid purpura nephritis in childhood: Natural history and immunopathology. Adv Nephrol Necker Hosp 1976;6:183-228.
13. Miyazaki M, Abe K, Koji T, Furusu A, Ozono Y, Harada T, et al. Intraglomerular C3 synthesis in human kidney detected by in situ hybridization. J Am Soc Nephrol 1996;7:2428-33.
14. Yüksel S, Çağlar M, Evrengül H, Becerir T, Tepeli E, Ergin A, et al. Could serum pentraxin 3 levels and IgM deposition in skin biopsies predict subsequent renal involvement in children with Henoch- Schönlein purpura? Pediatr Nephrol 2015;30:969-74.
15. Spitzer RE, Urmson JR, Farnett ML, Stitzel AE, Post EM. Alteration of the complement system in children with Henoch Schönlein purpura. Clin Immunol Immunopathol 1978;11:52-9.
16. al-Sheyyab M, Batieha A, el-Shanti H, Daoud A. Henoch-Schonlein purpura and streptococcal infection: A prospective case-control study. Ann Trop Paediatr 1999;19:253-5.
17. Urizar RE, Singh JK, Muhammad T, Hines O. Henoch-Schönlein anaphylactoid purpura nephropathy: Electron microscopic lesions mimicking acute poststreptococcal nephritis. Hum Pathol 1978;9:223-9.
18. Goodyer PR, de Chadarevian JP, Kaplan BS. Acute poststreptococcal glomerulonephritis mimicking Henoch-Schönlein purpura. J Pediatr 1978;93:412-5.
19. Kim CK, Aikawa M, Makker SP. Electron-dense subepithelial glomerular deposits in Henoch-Schönlein purpura syndrome. Arch Pathol Lab Med 1979;103:595-8.
20. Rivera F, Anaya S, Pérez-Alvarez J, de la Nieta MD, Vozmediano MC, Blanco J. Henoch-Schönlein nephritis associated with streptococcal infection and persistent hypocomplementemia: A case report. J Med Case Rep 2010;11;4:50.
21. Lin Q, Li X. Children with Henoch-Schönlein purpura with low complement levels: Follow-up for >6 years. Pediatr Nephrol 2017;32:1279.

Year 2019, Volume: 13 Issue: 3, 208 - 212, 24.05.2019

Özge Başaran

https://doi.org/10.12956/tjpd.2018.367

Abstract

References

1. McCarthy HJ, Tizard EJ. Clinical practice: Diagnosis and management of Henoch-Schönlein purpura. Eur J Pediatr 2010;169:643-50.
2. Ballinger S. Henoch-Schonlein purpura. Curr Opin Rheumatol 2003;15:591-4.
3. Lau KK, Suzuki H, Novak J, Wyatt RJ. Pathogenesis of Henoch- Schönlein purpura nephritis. Pediatr Nephrol 2010;25:19-26.
4. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013;65:1-11.
5. Hisano S, Matsushita M, Fujita T, Takeshita M, Iwasaki H. Activation of the lectin complement pathway in post-streptococcal acute glomerulonephritis. Pathol Int 2007;57:351-7.
6. Garcia-Fuentes M, Martin A, Chantler C, Williams DG. Serum complement components in Henoch-Schönlein purpura. Arch Dis Child 1978;53:417-9.
7. Islek I, Muslu A, Dagdemir A, Dilber C. Is low serum complement 3 in Henoch Schönlein purpura unusual and of prognostic value? Eur J Pediatr 2001;160:397-8.
8. Motoyama O, Iitaka K. Henoch-Schonlein purpura with hypocomplementemia in children. Pediatr Int 2005;47:39-42.
9. Lin Q, Min Y, Li Y, Zhu Y, Song X, Xu Q, et al. Henoch-Schönlein purpura with hypocomplementemia. Pediatr Nephrol 2012;27:801- 6.
10. Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. Paediatric Rheumatology International Trials Organisation (PRINTO). EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 2010;69:798- 806.
11. Krause I, Garty BZ, Davidovits M, Cleper R, Tamary H, Rosenmann E, et al. Low serum C3, leukopenia, and thrombocytopenia: Unusual features of henoch-schonlein purpura. Eur J Pediatr 1999;158:906-9.
12. Levy M, Broyer M, Arsan A, Levy-Bentolila D, Habib R. Anaphylactoid purpura nephritis in childhood: Natural history and immunopathology. Adv Nephrol Necker Hosp 1976;6:183-228.
13. Miyazaki M, Abe K, Koji T, Furusu A, Ozono Y, Harada T, et al. Intraglomerular C3 synthesis in human kidney detected by in situ hybridization. J Am Soc Nephrol 1996;7:2428-33.
14. Yüksel S, Çağlar M, Evrengül H, Becerir T, Tepeli E, Ergin A, et al. Could serum pentraxin 3 levels and IgM deposition in skin biopsies predict subsequent renal involvement in children with Henoch- Schönlein purpura? Pediatr Nephrol 2015;30:969-74.
15. Spitzer RE, Urmson JR, Farnett ML, Stitzel AE, Post EM. Alteration of the complement system in children with Henoch Schönlein purpura. Clin Immunol Immunopathol 1978;11:52-9.
16. al-Sheyyab M, Batieha A, el-Shanti H, Daoud A. Henoch-Schonlein purpura and streptococcal infection: A prospective case-control study. Ann Trop Paediatr 1999;19:253-5.
17. Urizar RE, Singh JK, Muhammad T, Hines O. Henoch-Schönlein anaphylactoid purpura nephropathy: Electron microscopic lesions mimicking acute poststreptococcal nephritis. Hum Pathol 1978;9:223-9.
18. Goodyer PR, de Chadarevian JP, Kaplan BS. Acute poststreptococcal glomerulonephritis mimicking Henoch-Schönlein purpura. J Pediatr 1978;93:412-5.
19. Kim CK, Aikawa M, Makker SP. Electron-dense subepithelial glomerular deposits in Henoch-Schönlein purpura syndrome. Arch Pathol Lab Med 1979;103:595-8.
20. Rivera F, Anaya S, Pérez-Alvarez J, de la Nieta MD, Vozmediano MC, Blanco J. Henoch-Schönlein nephritis associated with streptococcal infection and persistent hypocomplementemia: A case report. J Med Case Rep 2010;11;4:50.
21. Lin Q, Li X. Children with Henoch-Schönlein purpura with low complement levels: Follow-up for >6 years. Pediatr Nephrol 2017;32:1279.

There are 21 citations in total.

Details

Primary Language	Turkish
Subjects	Internal Diseases
Journal Section	ORIGINAL ARTICLES
Authors	Özge Başaran
Publication Date	May 24, 2019
Submission Date	January 9, 2018
Published in Issue	Year 2019 Volume: 13 Issue: 3

Cite

Vancouver	Başaran Ö. Düşük Kompleman Düzeyi Olan Henoch Schönlein Purpuralı Çocuk Hastaların Değerlendirilmesi. Türkiye Çocuk Hast Derg. 2019;13(3):208-12.

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