Dergiler / Respiratory Case Reports / 2021 / Cilt: 10 - Sayı: 3

A Rare Coexistence of Birt-Hogg-Dubé Syndrome and Sarcoidosis

Birt-Hogg-Dubé Sendromu ve Sarkoidozun Nadir Birlikteliği

Sayfa
186–189
DOI
—
🏛️Bu makalenin bibliyografik verileri ve tam metin bağlantısı DergiPark üzerinden sağlanmıştır.
DergiPark Kaydı ↗
✨

Yapay Zeka Hızlı Bakış & Çıkarımlar

AI Insight
⚡ TL;DR(1 Cümlede Hızlı Çıkarım)

Enlarged mediastinal lymphad- enopathy may be underdiagnosed in patients with BHD, and so both the mediastinal and lung window of a thoracic CT scan should be evaluated carefully and a biopsy should be performed if pathologic lym- phadenopathies are indicated.

📌 3 Maddede Bu Makale

🎯
Amaç & Hipotez:A pathophysiological link may exist between Birt- Hogg-Dubé syndrome (BHD) and pulmonary sar- coidosis such that the folliculin protein encoded by the FLCN gene may lead to the development of sar- coidosis through the upregulation of T- helper 1 cell activation.
📊
Temel Bulgular & Veri:We present here a rare case of coexisting BHD and sarcoidosis.
💡
Sonuç & Katkı:Enlarged mediastinal lymphad- enopathy may be underdiagnosed in patients with BHD, and so both the mediastinal and lung window of a thoracic CT scan should be evaluated carefully and a biopsy should be performed if pathologic lym- phadenopathies are indicated.
🔬Tür:Araştırma Makalesi
📍Kapsam:Genişletilmiş Akademik Örneklem
⚙️Değişkenler:Alan Parametreleri

Özet

A pathophysiological link may exist between Birt- Hogg-Dubé syndrome (BHD) and pulmonary sar- coidosis such that the folliculin protein encoded by the FLCN gene may lead to the development of sar- coidosis through the upregulation of T- helper 1 cell activation. We present here a rare case of coexisting BHD and sarcoidosis. Enlarged mediastinal lymphad- enopathy may be underdiagnosed in patients with BHD, and so both the mediastinal and lung window of a thoracic CT scan should be evaluated carefully and a biopsy should be performed if pathologic lym- phadenopathies are indicated.

Abstract

Birt-Hogg-Dube (BHD) sendromuna sahip hastalarda FLCN geni tarafından kodlanan folliculin proteininin T-helper-1 hücre aktivasyonuna yol açarak sarkoido- za yol açabileceği ve bu sebeple bu iki hastalık ara- sında patofizyolojik bir ortaklık olabileceği düşünül- mektedir. Burada nadir bir BHD sendromu ve sarkoi- doz birlikteliği olgusunu sunuyoruz. BHD tanısı alan hastaların toraks bilgisayarlı tomografilerinin hem parankim hem de mediasten penceresinde olası bir mediastinal lenfadenopatiyi gözden kaçırmamak adına detaylı olarak incelenemeli ve patolojik boyutta bir lenf nodu mevcut ise biyopsi yapılmalıdır

🔖

Bu Makaleye Atıf Yap

SATICI, C., KÖYBAŞI, G., KOYUKAN, A., SEYHAN, E. C. (2021). A Rare Coexistence of Birt-Hogg-Dubé Syndrome and Sarcoidosis. Respiratory Case Reports, Cilt: 10 - Sayı: 3, 186–189. https://ajindex.com/articles/413558

📄 Tam Metin PDF Okuyucu

📄

PDF görüntüleyici hazırlanıyor...

Kaynakça (1)

  1. Menko FH, van Steensel MA, Giraud S, Friis-Hansen L, Richard S, Ungari S, et al. European BHD Consortium. Birt-Hogg-Dubé syndrome: diagnosis and management. Lancet Oncol 2009; 10:1199-206. [CrossRef] 2. Daccord C, Good JM, Morren MA, Bonny O, Hohl D, Lazor R. Birt-Hogg-Dubé syndrome. Eur Respir Rev 2020; 29:200042. [CrossRef] 3. Kuhn B, Teckchandani P, Harper R. Parsimony or poor luck: Concurrent Birt-Hogg- Dubé syndrome and sarcoi- dosis. Sarcoidosis Vasc Diffuse Lung Dis 2017; 34:194-6. [CrossRef] 4. Gupta N, Sunwoo BY, Kotloff RM. Birt-Hogg-Dubé synd- rome. Clin Chest Med 2016; 37:475-86. [CrossRef] 5. Kumasaka T, Hayashi T, Mitani K, Kataoka H, Kikkawa M, Tobino K, et al. Characterization of pulmonary cysts in Birt-Hogg-Dubé syndrome: histopathological and morphometric analysis of 229 pulmonary cysts from 50 unrelated patients. Histopathology 2014; 65:100-10. [CrossRef]