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Primer Silyer Diskinezide Son Gelişmeler 2015/6 Prof. Dr. Bülent KARADAĞ Marmara Üni. Çocuk Göğüs Hast. BD

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Page 1: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility

Primer Silyer Diskinezide

Son Gelişmeler 2015/6

Prof. Dr. Bülent KARADAĞ

Marmara Üni. Çocuk Göğüs Hast. BD

Page 2: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility

Primer silyer diskinezi

• 2354 yayın

• 2015-6: 215

Page 3: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility

PCD

1. Tanı

2. Klinik

2. Yaşam kalitesi

3. Genetik

4. SFT

5. Tedavi

Page 4: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility
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Page 25: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility

Çeşitli tanısal testler

Her bir testin avantajı ve kısıtlı olduğu yönler var.

“Altın standart” bir test yok.

İleriderece uzmanlaşmış merkezler örnekleri çalışıp

sonucu değerlendirmeli.

Page 26: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility
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PCD

1. Tanı

2. Klinik

2. Yaşam kalitesi

3. Genetik

4. SFT

5. Tedavi

Page 35: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility
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Page 38: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility

Hastaların %39’u en az bir kez Pseudomonas kolonizasyonu

kriterlerini karşıladı.

Daha önce bildirilenden yüksek.

Page 39: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility
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PCD

1. Tanı

2. Klinik

2. Yaşam kalitesi

3. Genetik

4. SFT

5. Tedavi

Page 42: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility
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Page 45: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility

PCD

1. Tanı

2. Klinik

2. Yaşam kalitesi

3. Genetik

4. SFT

5. Tedavi

Page 46: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility
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Page 49: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility

PCD

1. Tanı

2. Klinik

2. Yaşam kalitesi

3. Genetik

4. SFT

5. Tedavi

Page 50: Primer Silyer Diskinezidedisease, ABPA Testing Unlikely PCD Abnormal Ciliary Structure and Function? Characteristic ciliary uttrastructural defects seen by electron (EM) Dysmotility
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PCD için kanıta dayalı tedavi YOK.

Önerilerin çoğu CF ve bronşiekteziden uyarlanmış.

PCD tedavisinin temel taşı, günlük solunum fizyoterapisi ile

havayolları temizliği ve infeksiyona bağlı alevlenmelerin antibiyotikle

ile tedavisi

Rutin takipte mutlaka olması gerekenler: SFT, balgam kültürü,

odyometrik testler, rutin aşılamalar (pnömokok ve influenza)

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www.pcdregistry.eu

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