Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon

Secil Conkar; Ebru Yılmaz; Kadriye Özdemir; İpek Kaplan Bulut; Halil Bozkaya; Sevgi Mir

TR EN

Hipertansiyon sebebi olarak Nörofibromatozis Tip 1(NF1)

Abstract

Nörofibromatozis tip 1 otozomal dominant geçiş gösteren nöroektodermal ve mesodermal dokuların displazisi sonucu ortaya çıkan klinik bulgular ile karekterizedir. Cafe-au-lait denilen karekteristik pigmente deri lezyonu, aksiller ve inguinal çillenme, deri nörofibromaları, Lisch nodülleri denilen pigmente iris hamartomaları, merkezi sinir sistemi tümörleri, periferik sinir sistemi tümörleri ve diğer sistemleri ilgilendiren tümörler görülebilir. Nörofibramatöz tip 1 de hipertansiyon renovasküler hastalıklar, mid-aortik sendrom veya feokromasitomaya bağlı olarak gelişebilir. Çocukluk yaşlarında nörofibramatöza bağlı hipertansiyonun nedeni genellikle renal arter stenozu olup genellikle damarın proksimalini veya orijini ilgilendirir. Hastaların %25’inde aort koarktasyonu ile birliktedir. Nörofibramatozis nadir görülen bir hastalık olsada çocukluk çağında ki renovasküler hipertansiyonun önemli nedenlerinden biridir. Nörofibramatöz tip 1 nedeniyle izlenirken hipertansiyon tespit edilen iki olgumuzu NF 1 izleminde kan basıncı ölçümleri ile tarama yapılmasının önemini belirtmek ve aort ana dallarının ve renal arterlerin hipertansiyon tespit edilen olgularda stenoz açısından incelenmesinin önemini vurgulamak için sunduk.

Keywords

nörofibromatozis, hipertansiyon, çocuk

Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon

Abstract

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder is characterized by various clinical manifestations as a result of dysplasia of neuroectodermal and mesodermal tissues. Cafe-au-lait spots, axillary and inguinal freckling, cutaneous neurofibromas with a variable clinical expression, iris Lisch nodules, and multiple tumors of central and peripheral nervous system, as well as other systems may be involved. NF1 patients develop hypertension due to renovascular diseases, mid-aortic syndrome, or pheochromocytoma. In pediatric NF1 patients elevated blood pressure is usually due to renal artery stenosis, generally involving the origin or the proximal tract of the vessel, and is associated in 25% of patients with coarctation of the abdominal aorta. Several patients with NF1 and hypertension have been reported in the literature, showing the extreme variability in anatomical lesions. Although NF1 is a rare disease, ıt is an important cause of renovasculer hypertension in childhood.Abdominal bruit is an alerting physical finding in diagnosis of renal artery stenosis. We present two cases with NF1 developed hypertension.Our cases emphasises the need for blood pressure measurement in children with NF1 screening for stenosis in the major branch vessels of the aorta in children with NF1 and renal artery stenosis is required in hypertensive NF1 patients.

Keywords

Neurofibromatosis, hypertension, children

References

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Details

Primary Language

English

Subjects

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Journal Section

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Authors

Secil Conkar This is me

Ebru Yılmaz This is me

Kadriye Özdemir This is me

İpek Kaplan Bulut This is me

Halil Bozkaya This is me

Sevgi Mir This is me

Publication Date

October 1, 2015

Submission Date

October 1, 2015

Acceptance Date

-

Published in Issue

Year 2015 Number: 3

IZ

https://izlik.org/JA86KB26MB

APA

Conkar, S., Yılmaz, E., Özdemir, K., Bulut, İ. K., Bozkaya, H., & Mir, S. (2015). Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon. Pamukkale Medical Journal, 3, 259-262. https://izlik.org/JA86KB26MB

AMA

1.Conkar S, Yılmaz E, Özdemir K, Bulut İK, Bozkaya H, Mir S. Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon. Pam Med J. 2015;(3):259-262. https://izlik.org/JA86KB26MB

Chicago

Conkar, Secil, Ebru Yılmaz, Kadriye Özdemir, İpek Kaplan Bulut, Halil Bozkaya, and Sevgi Mir. 2015. “Neurofıbromatosıs Type 1 (NF1): The Causes of Hypertensıon”. Pamukkale Medical Journal, nos. 3: 259-62. https://izlik.org/JA86KB26MB.

EndNote

Conkar S, Yılmaz E, Özdemir K, Bulut İK, Bozkaya H, Mir S (October 1, 2015) Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon. Pamukkale Medical Journal 3 259–262.

IEEE

[1]S. Conkar, E. Yılmaz, K. Özdemir, İ. K. Bulut, H. Bozkaya, and S. Mir, “Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon”, Pam Med J, no. 3, pp. 259–262, Oct. 2015, [Online]. Available: https://izlik.org/JA86KB26MB

ISNAD

Conkar, Secil - Yılmaz, Ebru - Özdemir, Kadriye - Bulut, İpek Kaplan - Bozkaya, Halil - Mir, Sevgi. “Neurofıbromatosıs Type 1 (NF1): The Causes of Hypertensıon”. Pamukkale Medical Journal. 3 (October 1, 2015): 259-262. https://izlik.org/JA86KB26MB.

JAMA

1.Conkar S, Yılmaz E, Özdemir K, Bulut İK, Bozkaya H, Mir S. Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon. Pam Med J. 2015;:259–262.

MLA

Conkar, Secil, et al. “Neurofıbromatosıs Type 1 (NF1): The Causes of Hypertensıon”. Pamukkale Medical Journal, no. 3, Oct. 2015, pp. 259-62, https://izlik.org/JA86KB26MB.

Vancouver

1.Secil Conkar, Ebru Yılmaz, Kadriye Özdemir, İpek Kaplan Bulut, Halil Bozkaya, Sevgi Mir. Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon. Pam Med J [Internet]. 2015 Oct. 1;(3):259-62. Available from: https://izlik.org/JA86KB26MB

Pamukkale Medical Journal is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY NC).

Hipertansiyon sebebi olarak Nörofibromatozis Tip 1(NF1)

Abstract

Keywords

Neurofıbromatosıs type 1 (NF1): The causes of hypertensıon

Abstract

Keywords

References

Details

Primary Language

Subjects

Journal Section

Authors

Publication Date

Submission Date

Acceptance Date

Published in Issue

IZ

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