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‫تالسيميا‬ ‫الفا‬
Alpha-Thalassemia
MAY6, 2014
:‫ليکنه‬
‫للا‬‫کليم‬
(
‫وردګ‬
)
:‫استاد‬ ‫الرښود‬
)‫زاده‬‫(امير‬‫زکريا‬ ‫ی‬‫پوهندو‬
1
‫الرحيم‬ ‫الرحمن‬ ‫للا‬ ‫بسم‬
‫تالسيميا‬‫الفا‬
‫پولي‬ ‫الفا‬ ‫د‬ ‫هيموګلوبينو‬ ‫د‬
‫عدم‬ ‫يا‬ ‫وجود‬ ‫د‬ ‫جينونو‬ ‫څلورو‬ ‫د‬ ‫ی‬‫دو‬ ‫د‬ ‫چې‬ ‫دی‬ ‫عبارت‬ ‫څخه‬ ‫نقص‬ ‫د‬ ‫زنځيرونو‬ ‫پيپتايد‬
‫کې‬ ‫شخص‬ ‫په‬ ‫اض‬‫ر‬‫اع‬ ‫کلينيکي‬ ‫ي‬‫ځانګړ‬ ‫ي‬ ‫ش‬ ‫کېدای‬ ‫يې‬ ‫يو‬ ‫هر‬ ‫چې‬ ‫ي‬‫کېږ‬ ‫بندی‬ ‫تصنيف‬ ‫ډولونو‬ ‫مختلفو‬ ‫په‬ ‫مخې‬ ‫له‬ ‫موجوديت‬
.‫ي‬‫کړ‬ ‫امنځته‬‫ر‬
‫الفا‬ ‫يې‬ ‫دوه‬ ‫څخه‬ ‫جملې‬ ‫له‬ ‫ګلوبوپروتينو‬ ‫څلورو‬ ‫د‬ ‫کې‬ ‫هيموګلوبينو‬ ‫په‬
‫جينونو‬ ‫څلورو‬ ‫د‬ ‫چې‬ ،‫ي‬‫و‬ ‫زنځيرونه‬ ‫پېپتايد‬ ‫پولي‬
‫جوړ‬‫(دوه‬‫څلورو‬‫ددې‬،‫ي‬‫توليديږ‬ ‫مخې‬‫له‬
‫ه‬
‫جوړه‬‫د‬‫ننه‬ ‫د‬‫ې‬‫حجر‬‫د‬‫موقعيتونه‬‫جينونو‬)
۱۶
‫چې‬،‫دي‬‫واقع‬‫پرسر‬‫کروموزومونو‬‫مو‬
.‫ې‬‫کړ‬ ‫احتوا‬ ‫جوړه‬ ‫يوه‬ ‫کروموزوم‬ ‫یوه‬ ‫هر‬
‫کېدای‬ ‫يعني‬ ،‫ي‬‫و‬ ‫ايګس‬‫ز‬‫هيترو‬ ‫يا‬ ‫ايګس‬‫ز‬‫هومو‬ ‫ي‬ ‫ش‬ ‫کېدای‬‫نقص‬ ‫جينونو‬ ‫نوموړو‬ ‫د‬
‫ي‬‫و‬ ‫مصاب‬ ‫کروموزوم‬ ‫يو‬ ‫ي‬‫يواز‬ ‫ي‬ ‫ش‬
‫دواړو‬ ‫د‬ ‫ياهم‬ ‫او‬ ‫ي‬‫ونلر‬ ‫وجود‬ ‫جينونه‬ ‫دواړه‬ ‫کروموزوم‬ ‫يوه‬ ‫د‬ ‫ي‬ ‫ش‬ ‫کېدای‬ ‫او‬ ،‫ي‬‫و‬ ‫ي‬‫شو‬ ‫مصاب‬ ‫کروموزومونه‬ ‫دواړه‬ ‫يا‬
:‫دي‬ ‫ي‬‫شو‬‫ويشل‬‫ډولونو‬ ‫څلورګونو‬ ‫الندنيو‬‫په‬ ‫تالسيميا‬ ‫الفا‬ ‫يې‬ ‫مخې‬ ‫له‬ ‫چې‬.‫ي‬‫و‬ ‫تللی‬ ‫منځه‬ ‫له‬ ‫جين‬ ‫يو‬ ‫يو‬ ‫کروموزومونو‬
۱
‫ناقل‬ ‫خاموش‬ .
‫حالت‬
(Silent Carrier State)
‫ي‬‫و‬‫خاموش‬ ‫جين‬ ‫يو‬ ‫چې‬‫کله‬:
.
۲
( ‫تالسيميا‬ ‫کوچنۍ‬.
Thalassemia Minor/Trait
‫جين‬ ‫دوه‬ ‫چي‬ ‫کله‬:)
‫ي‬‫ونلر‬‫وجود‬‫ونه‬
.
۳
‫د‬.
H
‫هيموګلوبين‬
‫مرض‬
(HbH Disease)
‫جين‬ ‫ی‬‫در‬ ‫چي‬ ‫کله‬ :
‫ي‬‫ونلر‬ ‫وجود‬ ‫ونه‬
.
۴
.
Bart’s Hydrops Fetalis Syndrome
‫ي‬‫ونلر‬ ‫وجود‬‫جينونه‬ ‫څلور‬ ‫چې‬ ‫کله‬ :
.
۱
:‫حالت‬ ‫ناقل‬ ‫خاموش‬.
•
‫وجود‬ ‫ې‬ ‫پکښ‬ ‫جينونه‬ ‫الفا‬ ‫فعاله‬ ‫ی‬‫در‬ ‫پاتې‬ ‫او‬ ،‫ي‬‫و‬ ‫تللی‬ ‫منځه‬ ‫له‬ ‫جين‬ ‫الفا‬ ‫يو‬ ‫کې‬ ‫حالت‬ ‫پدې‬
.‫ي‬‫لر‬
•
.‫ي‬‫و‬‫نارمل‬
ً
‫تقريبا‬ ‫نسبت‬ ‫زنځيرونو‬ ‫بيتا‬ ‫او‬ ‫الفا‬ ‫د‬
•
.‫موجوده‬ ‫ي‬‫و‬‫نه‬ ‫ابنارمليتی‬ ‫هيماتولوژيکه‬‫کومه‬
•
‫جنيتيکي‬ ‫د‬ ‫بايد‬ ،‫دی‬ ‫ستونزمن‬ ‫تشخيص‬ ‫حالت‬ ‫ددی‬ ‫ميتودونو‬ ‫هيماتولوژيکو‬ ‫په‬
mapping
.‫ي‬ ‫ش‬‫تشخيص‬ ‫وسیله‬‫په‬
•
‫حده‬ ‫يوه‬ ‫تر‬ ‫ي‬ ‫ش‬‫کېدای‬
MCV
.‫ي‬ ‫ش‬ ‫وليدل‬ ‫ټيټ‬
2
۲
‫تالسيميا‬ ‫الفا‬ ‫کوچنۍ‬.
:
•
‫يا‬ ‫ايګس‬‫ز‬‫هومو‬‫ي‬ ‫ش‬‫کیدای‬ ‫چي‬،‫ي‬‫کيږ‬‫امنځته‬‫ر‬ ‫وجهی‬‫له‬ ‫موجوديت‬ ‫عدم‬‫د‬ ‫جينونو‬ ‫الفا‬ ‫ددوو‬
.‫ي‬‫و‬‫ايګس‬‫ز‬‫هيترو‬
•
‫خفيفه‬
.‫ي‬‫کو‬‫امنځته‬‫ر‬ ‫انيميا‬ ‫هايپوکروميک‬،‫مايکروسايتيک‬
•
MCV
.‫ي‬‫و‬‫ټيټ‬ ‫ډېر‬
•
.‫ي‬ ‫ش‬ ‫مغالطه‬ ‫سره‬‫انيميا‬ ‫له‬ ‫کمبود‬ ‫د‬‫اوسپنې‬ ‫د‬ ‫ی‬ ‫ش‬‫کیدای‬
•
.‫موجود‬‫ی‬‫و‬‫نه‬‫کی‬‫کهولت‬‫په‬‫خو‬،‫ی‬‫و‬‫موجود‬‫اندازه‬‫څه‬‫په‬‫هيموګلوبين‬‫بارټ‬‫د‬‫پرمهال‬‫تولد‬‫د‬
۳
‫د‬.
H
:‫مرض‬ ‫هيموګلوبين‬
•
‫ترټول‬ ‫درجه‬ ‫دوهمه‬ ‫کی‬ ‫تالسيميا‬ ‫الفا‬ ‫په‬
.‫دی‬‫شکل‬ ‫شدید‬ ‫و‬
•
.‫ي‬‫مشخصيږ‬ ‫موجوديت‬ ‫پر‬ ‫جين‬ ‫فعاله‬ ‫يوه‬ ‫د‬ ‫ې‬‫يواز‬
•
‫بارټ‬ ‫د‬ ‫پرمهال‬ ‫تولد‬ ‫د‬ ‫چي‬ ،‫ي‬‫کيږ‬ ‫منجر‬ ‫اکم‬‫ر‬‫ت‬ ‫په‬ ‫زنځيرونو‬ ‫بیتا‬ ‫او‬ ‫ګاما‬ ‫مزدوجو‬ ‫غير‬ ‫اضافی‬ ‫د‬
‫هيموګلوبين‬
۱۰
-
۴۰
،‫ي‬‫و‬٪
‫تر‬ ‫مقدار‬ ‫هيموګلوبين‬ ‫ل‬‫ډو‬ ‫ددی‬ ‫کې‬ ‫کهولت‬‫په‬ ‫او‬
۵۰
.‫ي‬‫رسيږ‬
ً
‫تدريجا‬ ‫پوری‬ ٪
•
‫ی‬ ‫ش‬ ‫کیدای‬ ‫کیدل‬ ‫مواجه‬ ‫سره‬ ‫دواګانو‬ ‫اوکسيديتیف‬ ،‫حمل‬ ،‫انتانات‬ ‫خو‬ ،‫ي‬‫ژوندلر‬ ‫نارمل‬
‫هیموالی‬
.‫ی‬‫کړ‬ ‫امنځته‬‫ر‬ ‫ان‬‫ر‬‫بح‬ ‫تیک‬
•
‫ډير‬ ‫له‬ ‫او‬ ‫هايپوکروميک‬ ،‫مایکروسایتیک‬ ‫کرویات‬ ‫سره‬
Poikilocytosis
.‫ي‬‫و‬ ‫يوځای‬ ‫سره‬
‫متعدد‬
Target Cells
.‫ي‬‫کيږ‬ ‫ليدل‬
۴
.
Bart’s Hydrops Fetalis Syndrome
:
•
.‫ي‬‫نلر‬ ‫زنځير‬ ‫الفا‬ ‫فعال‬‫هيڅ‬.‫ي‬‫نکو‬ ‫توافق‬ ‫هيڅ‬ ‫سره‬‫ژوند‬ ‫له‬ .‫دی‬ ‫ل‬‫ډو‬ ‫شديد‬ ‫ډير‬
•
‫ماشوم‬
‫اکم‬‫ر‬‫ت‬‫له‬‫مايع‬‫مصلي‬‫د‬‫کې‬‫انساجو‬‫جنيني‬‫په‬‫چې‬‫کوم‬،‫ي‬‫زيږيږ‬‫يوځای‬‫سره‬‫حالت‬‫فټالس‬‫هايډوپس‬‫له‬
‫په‬ ‫انيميا‬ ‫شديدې‬ ‫د‬‫شکل‬ ‫په‬ ‫حبن‬ ‫او‬ ‫اذيما‬ ‫د‬‫څخه‬
‫کې‬ ‫نتيجه‬
‫کبدي‬ ‫همدارنګه‬.‫ي‬‫کېږ‬ ‫امنځته‬‫ر‬
-
‫قلبي‬ ‫او‬ ‫طحالي‬
‫هم‬ ‫ضخامه‬
‫ي‬‫و‬‫موجوده‬
.
•
‫پورټلنډ‬ ،‫ي‬‫و‬‫هيموګلوبينو‬ ‫د‬‫بارټ‬ ‫د‬ ‫فيصدي‬‫غالبه‬
Portland
‫او‬
H
.‫ي‬‫کېږ‬ ‫ليدل‬ ‫هم‬ ‫هيموګلوبين‬
•
‫يې‬ ‫ته‬ ‫انساجو‬ ‫کولی‬ ‫ي‬ ‫نش‬ ‫يې‬ ‫امله‬ ‫له‬ ‫چې‬ ‫ي‬‫جوړو‬ ‫اتصال‬ ‫ټينګ‬ ‫ډېر‬ ‫سره‬ ‫آکسيجنو‬ ‫له‬ ‫هيموګلوبين‬ ‫بارټ‬
‫او‬ ‫هايپوکروميک‬ ‫شديده‬ ‫جنين‬ ‫پرمهال‬ ‫تولد‬ ‫د‬ .‫ي‬‫مر‬ ‫تولده‬ ‫له‬ ‫وروسته‬ ‫لږ‬ ‫يا‬ ‫او‬ ‫کې‬ ‫رحم‬ ‫په‬ ‫جنين‬ .‫ي‬‫وسپار‬
.‫ي‬‫لر‬ ‫انيميا‬ ‫مايکروسايتيک‬
•
‫خط‬ ‫ته‬ ‫مور‬ ‫حمل‬ ‫ل‬‫ډو‬‫دا‬
.‫دی‬ ‫لوړ‬ ‫ډېر‬‫يې‬ ‫خطر‬ ‫نزف‬ ‫الوالدتي‬ ‫بعد‬ ‫او‬ ‫ټاکسيميا‬ ‫د‬،‫دی‬ ‫رناک‬
3
Alpha-Thalassemia with Hemoglobin S
:
‫هيموګلوبين‬‫له‬ ‫تالسيميا‬ ‫الفا‬
S
.‫دی‬ ‫ترکيب‬ ‫عام‬
ً
‫نسبتا‬ ‫يو‬ ‫کې‬ ‫اد‬‫ژ‬‫ن‬ ‫افريقايي‬ ‫په‬ ‫چې‬.‫ي‬ ‫ش‬‫پېښېدلی‬ ‫هم‬‫يوځای‬ ‫سره‬
‫د‬ ،‫ي‬‫و‬ ‫ي‬ ‫عرض‬ ‫غير‬
ً
‫معموال‬ ‫مريض‬
Sickle Cell
‫کمو‬ ‫د‬ ‫هم‬ ‫بيا‬ ‫نسبت‬ ‫په‬
HbS
‫د‬ ‫او‬ ،‫ي‬‫و‬ ‫لرونکی‬
HbF
‫ې‬ ‫پکښ‬ ‫مقدار‬ ‫لوړ‬
‫ي‬‫کېږ‬ ‫ليدل‬
.
➢
:‫معاينات‬ ‫البراتواري‬
:‫دی‬‫مهم‬ ‫ې‬ ‫پکښ‬ ‫هم‬‫اصل‬ ‫ادي‬‫ژ‬‫ن‬ ،‫ي‬ ‫ش‬‫ل‬‫ونيو‬ ‫کې‬ ‫نظر‬ ‫په‬ ‫وروسته‬‫څخه‬ ‫تاريخچې‬ ‫فاميلي‬ ‫او‬ ‫ي‬ ‫شخص‬‫له‬ ‫مريض‬ ‫د‬ ‫بايد‬
‫معاينات‬‫فزيکي‬‫خاطر‬‫په‬‫کولو‬ ‫پيدا‬‫د‬‫عالئمو‬ ‫الندې‬ ‫د‬ ‫بايد‬‫لومړی‬
:‫ي‬ ‫ش‬ ‫ترسره‬
•
.‫ي‬‫کو‬ ‫ښکارندويي‬‫کمخونۍ‬ ‫د‬‫چې‬‫خسافت‬
•
‫تجزيې‬ ‫د‬ ‫وينې‬ ‫د‬ ‫چې‬‫ی‬‫ژېړ‬
Hemolysis
‫ده‬‫نښه‬
•
.‫ي‬‫کېږ‬‫امنځته‬‫ر‬ ‫امله‬ ‫له‬ ‫تجمع‬ ‫غيرنورمالې‬ ‫د‬‫وينې‬‫د‬ ‫چې‬‫لويوالی‬ ‫طحال‬‫د‬
•
‫تالسيميا‬ ‫بيټا‬ ‫له‬
ُ
‫معموال‬ ‫چې‬‫شکل‬‫سوء‬‫اسکليټ‬ ‫د‬
Major
.‫ي‬‫کېږ‬‫امنځته‬‫ر‬ ‫څخه‬
✓
CBC
:‫سره‬‫تشخيصونو‬‫تفريقي‬ ‫له‬
‫هيموګلوب‬ ‫د‬
،‫هيماټوکريټ‬ ‫د‬ ،‫ينو‬
Mean Carpuscular Volume (MCV)
‫او‬ ،
Mean Carpuscular Hemoglobin
(MCH)
‫د‬ ‫مقدار‬ ‫ی‬‫شو‬ ‫کم‬ ‫يوڅه‬ ‫يا‬ ‫نارمل‬ ‫ښايي‬ ‫سقوط؛‬ ‫سويې‬ ‫د‬
Mean Carpuscular Hemoglobin Concentration
(MCHC)
.‫ي‬‫و‬‫غالب‬ ‫ې‬ ‫پکښ‬ ‫حالت‬ ‫هايپوکروميک‬ ‫او‬ ‫مايکروسايټيک‬ ‫ښايی‬.‫ي‬ ‫ش‬‫وليدل‬
Genotype HbA HbBart HbH
Normal 97-98% 0 0
Silent Carrier 96-98% 0-2% 0
Alpha Thalassemia Trait 85-95% 5-10% 0
HemoglobinHDisease Dec 25-40% 2-40%
Hydrops Fetalis 0
80% (with20%
HgbPortland)
0-20%
4
‫او‬ ‫لوړ‬ ‫ممکن‬ ‫تعداد‬ ‫کروياتو‬ ‫سرو‬ ‫د‬
MCV
‫سره‬ ‫کمخونۍ‬ ‫له‬ ‫کمبود‬ ‫د‬ ‫اوسپنې‬ ‫د‬ ‫تالسيميا‬ ‫يې‬ ‫مخې‬ ‫له‬ ‫چې‬ ،‫ي‬‫و‬ ‫ټيټ‬
.‫ي‬‫تفريقيږ‬
.‫ي‬‫کېږ‬ ‫ليدل‬ )‫حالت‬ ‫ناقل‬ ‫خاموش‬ ‫د‬ ‫استثناء‬ ‫(په‬ ‫کرويات‬ ‫سره‬ ‫هايپوکروميک‬،‫مايکروسايتيک‬ ،‫کې‬‫تشخيص‬ ‫تفريقي‬ ‫په‬
‫متوسط‬ ‫يا‬ ‫خفيف‬ ‫ي‬ ‫ش‬ ‫کېدای‬
Poikilocytosis
‫ش‬ ‫وليدل‬ ‫ې‬ ‫پکښ‬
‫د‬ ‫ي‬ ‫ش‬ ‫کېدای‬ ‫کې‬ ‫حاالتو‬ ‫شديدو‬ ‫ډېرو‬ ‫په‬ .‫ي‬
Elliptocytes and
Target Cells
.‫ي‬ ‫ش‬ ‫وليدل‬ ‫تعداد‬ ‫لوړ‬ ‫يو‬
‫همدارنګه‬
Polychromasia
‫او‬
BasophilicStippling
.‫ي‬ ‫ش‬ ‫کېدی‬ ‫ليدل‬ ‫ې‬ ‫پکښ‬
✓
Reticulocyte Count
:
.‫ي‬‫و‬ ‫تړلی‬ ‫پورې‬ ‫شدته‬‫تر‬ ‫تالسيميا‬ ‫د‬‫شدت‬‫لوړوالي‬ ‫ددې‬ ‫چې‬،‫ي‬‫و‬‫لوړ‬ ‫مقدار‬ ‫يې‬
ُ
‫معموال‬
✓
Routine ChemistryTests
:
Indirect Bilirubin
‫متوسطه‬ ‫او‬ ‫لويه‬ ‫په‬ ‫ښايي‬
(MajorandIntermedia)
‫تالسيميا‬
.‫ي‬ ‫ش‬ ‫وليدل‬ ‫کې‬
، ‫سويه‬ ‫اوسپنې‬ ‫د‬
Total binding Capacity
‫او‬
FerritinLevel
.‫ي‬ ‫ش‬ ‫بېلولی‬ ‫کمخونيو‬‫له‬ ‫کمبود‬ ‫د‬‫اوسپنې‬ ‫د‬‫تالسيميا‬
✓
:‫عمليې‬ ‫ځانګړې‬‫نورې‬
‫د‬ ‫ګلوبين‬‫د‬
.‫ي‬‫کو‬‫ښودنه‬ ‫نسبت‬ ‫د‬‫ترمنځ‬ ‫زنځيرونو‬ ‫ګلوبين‬ ‫شويو‬‫توليد‬ ‫د‬‫چې‬‫ټسټونه؛‬ ‫زنځير‬
.‫ي‬‫ممکنو‬ ‫پېژندنه‬‫نقص‬ ‫جنيټيکي‬ ‫مشخص‬ ‫د‬ ‫چې‬ ‫تحليل؛‬ ‫اې‬ ‫ان‬ ‫ډي‬‫د‬
❖
Management
:
‫د‬
Alpha-thalassemia Trait
‫او‬
B-thalassemia Minor
.‫ي‬‫کېږ‬ ‫ليدل‬ ‫نه‬ ‫ضرورت‬ ‫ته‬ ‫ۍ‬‫تداو‬ ‫کومې‬ ‫هېڅ‬ ‫لپاره‬
‫ې‬‫يواز‬
.‫ي‬ ‫وش‬ ‫ي‬‫مخنيو‬ ‫سرګردانۍ‬‫له‬ ‫مريض‬ ‫د‬‫ترڅو‬ ‫ده‬ ‫ي‬‫ضرور‬ ‫يې‬ ‫پېژندنه‬
‫د‬
Hemoglobin H Disease
‫د‬ ‫بايد‬ ‫او‬ ،‫ده‬ ‫مفيده‬ )‫ې‬‫الر‬ ‫له‬ ‫خولې‬ ‫د‬ ‫کې‬ ‫ورځ‬ ‫په‬ ‫ام‬‫ر‬‫ګ‬ ‫(يو‬ ‫توصيه‬ ‫اسيډو‬ ‫فوليک‬ ‫د‬ ‫لپاره‬
OxidativeDrugs
.‫ي‬ ‫وش‬ ‫ی‬‫مخنيو‬ ‫څخه‬ ‫استعمال‬ ‫له‬
‫اسيدو‬ ‫فوليک‬ ‫د‬ ‫بايد‬ ‫او‬ ‫ي‬ ‫ش‬ ‫تطبيق‬ ‫الدم‬‫نقل‬ ‫مخې‬ ‫له‬ ‫اوقات‬ ‫تقسيم‬ ‫منظم‬ ‫يوه‬ ‫د‬‫بايد‬ ‫ته‬ ‫مريضانو‬ ‫تالسيميا‬ ‫شديدې‬ ‫د‬
.‫واخلي‬‫ات‬‫ر‬‫مستحض‬
.‫ي‬‫کېږ‬ ‫ترسره‬ ‫کې‬‫صورت‬ ‫په‬ ‫تخريب‬ ‫دوامداره‬ ‫د‬ ‫وينې‬ ‫د‬‫وجه‬‫په‬ ‫هايپرسپلينيزم‬ ‫د‬ ‫ې‬‫يواز‬ ‫سلينيکټومي‬
5
‫ش‬‫ليدل‬ ‫ندی‬‫تسممم‬‫اعضاوو‬ ‫د‬‫يې‬‫اوسه‬‫ر‬‫ت‬‫چې‬‫ماشومان‬‫هغه‬
‫له‬‫او‬،‫ي‬‫لر‬ ‫ژوند‬‫ښه‬‫ی‬‫و‬
۸۰
‫کلونو‬‫ډېرو‬‫تر‬ ‫يې‬‫ډېر‬‫څخه‬٪
.‫پايي‬ ‫پورې‬
:‫مأخذونه‬
1. Harrison principles of internal medicine, 17th edition
2. Cecilmedicine, 23rd Edition
3. The big picture pathology, Walter l. Kemp
4. Basic Medical Biochemistry, aclinical Approach
5. Black’sMedical Dictionary, 41st edition
6. Dr.Najib’s lectures
‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬
‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬
‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬
‫التوفيق‬ ‫للا‬‫من‬‫و‬
10:50 pm/12/May/2014—‫دوشنبه‬
Kandahar, Afghanistan
‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬
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  • 2. 1 ‫الرحيم‬ ‫الرحمن‬ ‫للا‬ ‫بسم‬ ‫تالسيميا‬‫الفا‬ ‫پولي‬ ‫الفا‬ ‫د‬ ‫هيموګلوبينو‬ ‫د‬ ‫عدم‬ ‫يا‬ ‫وجود‬ ‫د‬ ‫جينونو‬ ‫څلورو‬ ‫د‬ ‫ی‬‫دو‬ ‫د‬ ‫چې‬ ‫دی‬ ‫عبارت‬ ‫څخه‬ ‫نقص‬ ‫د‬ ‫زنځيرونو‬ ‫پيپتايد‬ ‫کې‬ ‫شخص‬ ‫په‬ ‫اض‬‫ر‬‫اع‬ ‫کلينيکي‬ ‫ي‬‫ځانګړ‬ ‫ي‬ ‫ش‬ ‫کېدای‬ ‫يې‬ ‫يو‬ ‫هر‬ ‫چې‬ ‫ي‬‫کېږ‬ ‫بندی‬ ‫تصنيف‬ ‫ډولونو‬ ‫مختلفو‬ ‫په‬ ‫مخې‬ ‫له‬ ‫موجوديت‬ .‫ي‬‫کړ‬ ‫امنځته‬‫ر‬ ‫الفا‬ ‫يې‬ ‫دوه‬ ‫څخه‬ ‫جملې‬ ‫له‬ ‫ګلوبوپروتينو‬ ‫څلورو‬ ‫د‬ ‫کې‬ ‫هيموګلوبينو‬ ‫په‬ ‫جينونو‬ ‫څلورو‬ ‫د‬ ‫چې‬ ،‫ي‬‫و‬ ‫زنځيرونه‬ ‫پېپتايد‬ ‫پولي‬ ‫جوړ‬‫(دوه‬‫څلورو‬‫ددې‬،‫ي‬‫توليديږ‬ ‫مخې‬‫له‬ ‫ه‬ ‫جوړه‬‫د‬‫ننه‬ ‫د‬‫ې‬‫حجر‬‫د‬‫موقعيتونه‬‫جينونو‬) ۱۶ ‫چې‬،‫دي‬‫واقع‬‫پرسر‬‫کروموزومونو‬‫مو‬ .‫ې‬‫کړ‬ ‫احتوا‬ ‫جوړه‬ ‫يوه‬ ‫کروموزوم‬ ‫یوه‬ ‫هر‬ ‫کېدای‬ ‫يعني‬ ،‫ي‬‫و‬ ‫ايګس‬‫ز‬‫هيترو‬ ‫يا‬ ‫ايګس‬‫ز‬‫هومو‬ ‫ي‬ ‫ش‬ ‫کېدای‬‫نقص‬ ‫جينونو‬ ‫نوموړو‬ ‫د‬ ‫ي‬‫و‬ ‫مصاب‬ ‫کروموزوم‬ ‫يو‬ ‫ي‬‫يواز‬ ‫ي‬ ‫ش‬ ‫دواړو‬ ‫د‬ ‫ياهم‬ ‫او‬ ‫ي‬‫ونلر‬ ‫وجود‬ ‫جينونه‬ ‫دواړه‬ ‫کروموزوم‬ ‫يوه‬ ‫د‬ ‫ي‬ ‫ش‬ ‫کېدای‬ ‫او‬ ،‫ي‬‫و‬ ‫ي‬‫شو‬ ‫مصاب‬ ‫کروموزومونه‬ ‫دواړه‬ ‫يا‬ :‫دي‬ ‫ي‬‫شو‬‫ويشل‬‫ډولونو‬ ‫څلورګونو‬ ‫الندنيو‬‫په‬ ‫تالسيميا‬ ‫الفا‬ ‫يې‬ ‫مخې‬ ‫له‬ ‫چې‬.‫ي‬‫و‬ ‫تللی‬ ‫منځه‬ ‫له‬ ‫جين‬ ‫يو‬ ‫يو‬ ‫کروموزومونو‬ ۱ ‫ناقل‬ ‫خاموش‬ . ‫حالت‬ (Silent Carrier State) ‫ي‬‫و‬‫خاموش‬ ‫جين‬ ‫يو‬ ‫چې‬‫کله‬: . ۲ ( ‫تالسيميا‬ ‫کوچنۍ‬. Thalassemia Minor/Trait ‫جين‬ ‫دوه‬ ‫چي‬ ‫کله‬:) ‫ي‬‫ونلر‬‫وجود‬‫ونه‬ . ۳ ‫د‬. H ‫هيموګلوبين‬ ‫مرض‬ (HbH Disease) ‫جين‬ ‫ی‬‫در‬ ‫چي‬ ‫کله‬ : ‫ي‬‫ونلر‬ ‫وجود‬ ‫ونه‬ . ۴ . Bart’s Hydrops Fetalis Syndrome ‫ي‬‫ونلر‬ ‫وجود‬‫جينونه‬ ‫څلور‬ ‫چې‬ ‫کله‬ : . ۱ :‫حالت‬ ‫ناقل‬ ‫خاموش‬. • ‫وجود‬ ‫ې‬ ‫پکښ‬ ‫جينونه‬ ‫الفا‬ ‫فعاله‬ ‫ی‬‫در‬ ‫پاتې‬ ‫او‬ ،‫ي‬‫و‬ ‫تللی‬ ‫منځه‬ ‫له‬ ‫جين‬ ‫الفا‬ ‫يو‬ ‫کې‬ ‫حالت‬ ‫پدې‬ .‫ي‬‫لر‬ • .‫ي‬‫و‬‫نارمل‬ ً ‫تقريبا‬ ‫نسبت‬ ‫زنځيرونو‬ ‫بيتا‬ ‫او‬ ‫الفا‬ ‫د‬ • .‫موجوده‬ ‫ي‬‫و‬‫نه‬ ‫ابنارمليتی‬ ‫هيماتولوژيکه‬‫کومه‬ • ‫جنيتيکي‬ ‫د‬ ‫بايد‬ ،‫دی‬ ‫ستونزمن‬ ‫تشخيص‬ ‫حالت‬ ‫ددی‬ ‫ميتودونو‬ ‫هيماتولوژيکو‬ ‫په‬ mapping .‫ي‬ ‫ش‬‫تشخيص‬ ‫وسیله‬‫په‬ • ‫حده‬ ‫يوه‬ ‫تر‬ ‫ي‬ ‫ش‬‫کېدای‬ MCV .‫ي‬ ‫ش‬ ‫وليدل‬ ‫ټيټ‬
  • 3. 2 ۲ ‫تالسيميا‬ ‫الفا‬ ‫کوچنۍ‬. : • ‫يا‬ ‫ايګس‬‫ز‬‫هومو‬‫ي‬ ‫ش‬‫کیدای‬ ‫چي‬،‫ي‬‫کيږ‬‫امنځته‬‫ر‬ ‫وجهی‬‫له‬ ‫موجوديت‬ ‫عدم‬‫د‬ ‫جينونو‬ ‫الفا‬ ‫ددوو‬ .‫ي‬‫و‬‫ايګس‬‫ز‬‫هيترو‬ • ‫خفيفه‬ .‫ي‬‫کو‬‫امنځته‬‫ر‬ ‫انيميا‬ ‫هايپوکروميک‬،‫مايکروسايتيک‬ • MCV .‫ي‬‫و‬‫ټيټ‬ ‫ډېر‬ • .‫ي‬ ‫ش‬ ‫مغالطه‬ ‫سره‬‫انيميا‬ ‫له‬ ‫کمبود‬ ‫د‬‫اوسپنې‬ ‫د‬ ‫ی‬ ‫ش‬‫کیدای‬ • .‫موجود‬‫ی‬‫و‬‫نه‬‫کی‬‫کهولت‬‫په‬‫خو‬،‫ی‬‫و‬‫موجود‬‫اندازه‬‫څه‬‫په‬‫هيموګلوبين‬‫بارټ‬‫د‬‫پرمهال‬‫تولد‬‫د‬ ۳ ‫د‬. H :‫مرض‬ ‫هيموګلوبين‬ • ‫ترټول‬ ‫درجه‬ ‫دوهمه‬ ‫کی‬ ‫تالسيميا‬ ‫الفا‬ ‫په‬ .‫دی‬‫شکل‬ ‫شدید‬ ‫و‬ • .‫ي‬‫مشخصيږ‬ ‫موجوديت‬ ‫پر‬ ‫جين‬ ‫فعاله‬ ‫يوه‬ ‫د‬ ‫ې‬‫يواز‬ • ‫بارټ‬ ‫د‬ ‫پرمهال‬ ‫تولد‬ ‫د‬ ‫چي‬ ،‫ي‬‫کيږ‬ ‫منجر‬ ‫اکم‬‫ر‬‫ت‬ ‫په‬ ‫زنځيرونو‬ ‫بیتا‬ ‫او‬ ‫ګاما‬ ‫مزدوجو‬ ‫غير‬ ‫اضافی‬ ‫د‬ ‫هيموګلوبين‬ ۱۰ - ۴۰ ،‫ي‬‫و‬٪ ‫تر‬ ‫مقدار‬ ‫هيموګلوبين‬ ‫ل‬‫ډو‬ ‫ددی‬ ‫کې‬ ‫کهولت‬‫په‬ ‫او‬ ۵۰ .‫ي‬‫رسيږ‬ ً ‫تدريجا‬ ‫پوری‬ ٪ • ‫ی‬ ‫ش‬ ‫کیدای‬ ‫کیدل‬ ‫مواجه‬ ‫سره‬ ‫دواګانو‬ ‫اوکسيديتیف‬ ،‫حمل‬ ،‫انتانات‬ ‫خو‬ ،‫ي‬‫ژوندلر‬ ‫نارمل‬ ‫هیموالی‬ .‫ی‬‫کړ‬ ‫امنځته‬‫ر‬ ‫ان‬‫ر‬‫بح‬ ‫تیک‬ • ‫ډير‬ ‫له‬ ‫او‬ ‫هايپوکروميک‬ ،‫مایکروسایتیک‬ ‫کرویات‬ ‫سره‬ Poikilocytosis .‫ي‬‫و‬ ‫يوځای‬ ‫سره‬ ‫متعدد‬ Target Cells .‫ي‬‫کيږ‬ ‫ليدل‬ ۴ . Bart’s Hydrops Fetalis Syndrome : • .‫ي‬‫نلر‬ ‫زنځير‬ ‫الفا‬ ‫فعال‬‫هيڅ‬.‫ي‬‫نکو‬ ‫توافق‬ ‫هيڅ‬ ‫سره‬‫ژوند‬ ‫له‬ .‫دی‬ ‫ل‬‫ډو‬ ‫شديد‬ ‫ډير‬ • ‫ماشوم‬ ‫اکم‬‫ر‬‫ت‬‫له‬‫مايع‬‫مصلي‬‫د‬‫کې‬‫انساجو‬‫جنيني‬‫په‬‫چې‬‫کوم‬،‫ي‬‫زيږيږ‬‫يوځای‬‫سره‬‫حالت‬‫فټالس‬‫هايډوپس‬‫له‬ ‫په‬ ‫انيميا‬ ‫شديدې‬ ‫د‬‫شکل‬ ‫په‬ ‫حبن‬ ‫او‬ ‫اذيما‬ ‫د‬‫څخه‬ ‫کې‬ ‫نتيجه‬ ‫کبدي‬ ‫همدارنګه‬.‫ي‬‫کېږ‬ ‫امنځته‬‫ر‬ - ‫قلبي‬ ‫او‬ ‫طحالي‬ ‫هم‬ ‫ضخامه‬ ‫ي‬‫و‬‫موجوده‬ . • ‫پورټلنډ‬ ،‫ي‬‫و‬‫هيموګلوبينو‬ ‫د‬‫بارټ‬ ‫د‬ ‫فيصدي‬‫غالبه‬ Portland ‫او‬ H .‫ي‬‫کېږ‬ ‫ليدل‬ ‫هم‬ ‫هيموګلوبين‬ • ‫يې‬ ‫ته‬ ‫انساجو‬ ‫کولی‬ ‫ي‬ ‫نش‬ ‫يې‬ ‫امله‬ ‫له‬ ‫چې‬ ‫ي‬‫جوړو‬ ‫اتصال‬ ‫ټينګ‬ ‫ډېر‬ ‫سره‬ ‫آکسيجنو‬ ‫له‬ ‫هيموګلوبين‬ ‫بارټ‬ ‫او‬ ‫هايپوکروميک‬ ‫شديده‬ ‫جنين‬ ‫پرمهال‬ ‫تولد‬ ‫د‬ .‫ي‬‫مر‬ ‫تولده‬ ‫له‬ ‫وروسته‬ ‫لږ‬ ‫يا‬ ‫او‬ ‫کې‬ ‫رحم‬ ‫په‬ ‫جنين‬ .‫ي‬‫وسپار‬ .‫ي‬‫لر‬ ‫انيميا‬ ‫مايکروسايتيک‬ • ‫خط‬ ‫ته‬ ‫مور‬ ‫حمل‬ ‫ل‬‫ډو‬‫دا‬ .‫دی‬ ‫لوړ‬ ‫ډېر‬‫يې‬ ‫خطر‬ ‫نزف‬ ‫الوالدتي‬ ‫بعد‬ ‫او‬ ‫ټاکسيميا‬ ‫د‬،‫دی‬ ‫رناک‬
  • 4. 3 Alpha-Thalassemia with Hemoglobin S : ‫هيموګلوبين‬‫له‬ ‫تالسيميا‬ ‫الفا‬ S .‫دی‬ ‫ترکيب‬ ‫عام‬ ً ‫نسبتا‬ ‫يو‬ ‫کې‬ ‫اد‬‫ژ‬‫ن‬ ‫افريقايي‬ ‫په‬ ‫چې‬.‫ي‬ ‫ش‬‫پېښېدلی‬ ‫هم‬‫يوځای‬ ‫سره‬ ‫د‬ ،‫ي‬‫و‬ ‫ي‬ ‫عرض‬ ‫غير‬ ً ‫معموال‬ ‫مريض‬ Sickle Cell ‫کمو‬ ‫د‬ ‫هم‬ ‫بيا‬ ‫نسبت‬ ‫په‬ HbS ‫د‬ ‫او‬ ،‫ي‬‫و‬ ‫لرونکی‬ HbF ‫ې‬ ‫پکښ‬ ‫مقدار‬ ‫لوړ‬ ‫ي‬‫کېږ‬ ‫ليدل‬ . ➢ :‫معاينات‬ ‫البراتواري‬ :‫دی‬‫مهم‬ ‫ې‬ ‫پکښ‬ ‫هم‬‫اصل‬ ‫ادي‬‫ژ‬‫ن‬ ،‫ي‬ ‫ش‬‫ل‬‫ونيو‬ ‫کې‬ ‫نظر‬ ‫په‬ ‫وروسته‬‫څخه‬ ‫تاريخچې‬ ‫فاميلي‬ ‫او‬ ‫ي‬ ‫شخص‬‫له‬ ‫مريض‬ ‫د‬ ‫بايد‬ ‫معاينات‬‫فزيکي‬‫خاطر‬‫په‬‫کولو‬ ‫پيدا‬‫د‬‫عالئمو‬ ‫الندې‬ ‫د‬ ‫بايد‬‫لومړی‬ :‫ي‬ ‫ش‬ ‫ترسره‬ • .‫ي‬‫کو‬ ‫ښکارندويي‬‫کمخونۍ‬ ‫د‬‫چې‬‫خسافت‬ • ‫تجزيې‬ ‫د‬ ‫وينې‬ ‫د‬ ‫چې‬‫ی‬‫ژېړ‬ Hemolysis ‫ده‬‫نښه‬ • .‫ي‬‫کېږ‬‫امنځته‬‫ر‬ ‫امله‬ ‫له‬ ‫تجمع‬ ‫غيرنورمالې‬ ‫د‬‫وينې‬‫د‬ ‫چې‬‫لويوالی‬ ‫طحال‬‫د‬ • ‫تالسيميا‬ ‫بيټا‬ ‫له‬ ُ ‫معموال‬ ‫چې‬‫شکل‬‫سوء‬‫اسکليټ‬ ‫د‬ Major .‫ي‬‫کېږ‬‫امنځته‬‫ر‬ ‫څخه‬ ✓ CBC :‫سره‬‫تشخيصونو‬‫تفريقي‬ ‫له‬ ‫هيموګلوب‬ ‫د‬ ،‫هيماټوکريټ‬ ‫د‬ ،‫ينو‬ Mean Carpuscular Volume (MCV) ‫او‬ ، Mean Carpuscular Hemoglobin (MCH) ‫د‬ ‫مقدار‬ ‫ی‬‫شو‬ ‫کم‬ ‫يوڅه‬ ‫يا‬ ‫نارمل‬ ‫ښايي‬ ‫سقوط؛‬ ‫سويې‬ ‫د‬ Mean Carpuscular Hemoglobin Concentration (MCHC) .‫ي‬‫و‬‫غالب‬ ‫ې‬ ‫پکښ‬ ‫حالت‬ ‫هايپوکروميک‬ ‫او‬ ‫مايکروسايټيک‬ ‫ښايی‬.‫ي‬ ‫ش‬‫وليدل‬ Genotype HbA HbBart HbH Normal 97-98% 0 0 Silent Carrier 96-98% 0-2% 0 Alpha Thalassemia Trait 85-95% 5-10% 0 HemoglobinHDisease Dec 25-40% 2-40% Hydrops Fetalis 0 80% (with20% HgbPortland) 0-20%
  • 5. 4 ‫او‬ ‫لوړ‬ ‫ممکن‬ ‫تعداد‬ ‫کروياتو‬ ‫سرو‬ ‫د‬ MCV ‫سره‬ ‫کمخونۍ‬ ‫له‬ ‫کمبود‬ ‫د‬ ‫اوسپنې‬ ‫د‬ ‫تالسيميا‬ ‫يې‬ ‫مخې‬ ‫له‬ ‫چې‬ ،‫ي‬‫و‬ ‫ټيټ‬ .‫ي‬‫تفريقيږ‬ .‫ي‬‫کېږ‬ ‫ليدل‬ )‫حالت‬ ‫ناقل‬ ‫خاموش‬ ‫د‬ ‫استثناء‬ ‫(په‬ ‫کرويات‬ ‫سره‬ ‫هايپوکروميک‬،‫مايکروسايتيک‬ ،‫کې‬‫تشخيص‬ ‫تفريقي‬ ‫په‬ ‫متوسط‬ ‫يا‬ ‫خفيف‬ ‫ي‬ ‫ش‬ ‫کېدای‬ Poikilocytosis ‫ش‬ ‫وليدل‬ ‫ې‬ ‫پکښ‬ ‫د‬ ‫ي‬ ‫ش‬ ‫کېدای‬ ‫کې‬ ‫حاالتو‬ ‫شديدو‬ ‫ډېرو‬ ‫په‬ .‫ي‬ Elliptocytes and Target Cells .‫ي‬ ‫ش‬ ‫وليدل‬ ‫تعداد‬ ‫لوړ‬ ‫يو‬ ‫همدارنګه‬ Polychromasia ‫او‬ BasophilicStippling .‫ي‬ ‫ش‬ ‫کېدی‬ ‫ليدل‬ ‫ې‬ ‫پکښ‬ ✓ Reticulocyte Count : .‫ي‬‫و‬ ‫تړلی‬ ‫پورې‬ ‫شدته‬‫تر‬ ‫تالسيميا‬ ‫د‬‫شدت‬‫لوړوالي‬ ‫ددې‬ ‫چې‬،‫ي‬‫و‬‫لوړ‬ ‫مقدار‬ ‫يې‬ ُ ‫معموال‬ ✓ Routine ChemistryTests : Indirect Bilirubin ‫متوسطه‬ ‫او‬ ‫لويه‬ ‫په‬ ‫ښايي‬ (MajorandIntermedia) ‫تالسيميا‬ .‫ي‬ ‫ش‬ ‫وليدل‬ ‫کې‬ ، ‫سويه‬ ‫اوسپنې‬ ‫د‬ Total binding Capacity ‫او‬ FerritinLevel .‫ي‬ ‫ش‬ ‫بېلولی‬ ‫کمخونيو‬‫له‬ ‫کمبود‬ ‫د‬‫اوسپنې‬ ‫د‬‫تالسيميا‬ ✓ :‫عمليې‬ ‫ځانګړې‬‫نورې‬ ‫د‬ ‫ګلوبين‬‫د‬ .‫ي‬‫کو‬‫ښودنه‬ ‫نسبت‬ ‫د‬‫ترمنځ‬ ‫زنځيرونو‬ ‫ګلوبين‬ ‫شويو‬‫توليد‬ ‫د‬‫چې‬‫ټسټونه؛‬ ‫زنځير‬ .‫ي‬‫ممکنو‬ ‫پېژندنه‬‫نقص‬ ‫جنيټيکي‬ ‫مشخص‬ ‫د‬ ‫چې‬ ‫تحليل؛‬ ‫اې‬ ‫ان‬ ‫ډي‬‫د‬ ❖ Management : ‫د‬ Alpha-thalassemia Trait ‫او‬ B-thalassemia Minor .‫ي‬‫کېږ‬ ‫ليدل‬ ‫نه‬ ‫ضرورت‬ ‫ته‬ ‫ۍ‬‫تداو‬ ‫کومې‬ ‫هېڅ‬ ‫لپاره‬ ‫ې‬‫يواز‬ .‫ي‬ ‫وش‬ ‫ي‬‫مخنيو‬ ‫سرګردانۍ‬‫له‬ ‫مريض‬ ‫د‬‫ترڅو‬ ‫ده‬ ‫ي‬‫ضرور‬ ‫يې‬ ‫پېژندنه‬ ‫د‬ Hemoglobin H Disease ‫د‬ ‫بايد‬ ‫او‬ ،‫ده‬ ‫مفيده‬ )‫ې‬‫الر‬ ‫له‬ ‫خولې‬ ‫د‬ ‫کې‬ ‫ورځ‬ ‫په‬ ‫ام‬‫ر‬‫ګ‬ ‫(يو‬ ‫توصيه‬ ‫اسيډو‬ ‫فوليک‬ ‫د‬ ‫لپاره‬ OxidativeDrugs .‫ي‬ ‫وش‬ ‫ی‬‫مخنيو‬ ‫څخه‬ ‫استعمال‬ ‫له‬ ‫اسيدو‬ ‫فوليک‬ ‫د‬ ‫بايد‬ ‫او‬ ‫ي‬ ‫ش‬ ‫تطبيق‬ ‫الدم‬‫نقل‬ ‫مخې‬ ‫له‬ ‫اوقات‬ ‫تقسيم‬ ‫منظم‬ ‫يوه‬ ‫د‬‫بايد‬ ‫ته‬ ‫مريضانو‬ ‫تالسيميا‬ ‫شديدې‬ ‫د‬ .‫واخلي‬‫ات‬‫ر‬‫مستحض‬ .‫ي‬‫کېږ‬ ‫ترسره‬ ‫کې‬‫صورت‬ ‫په‬ ‫تخريب‬ ‫دوامداره‬ ‫د‬ ‫وينې‬ ‫د‬‫وجه‬‫په‬ ‫هايپرسپلينيزم‬ ‫د‬ ‫ې‬‫يواز‬ ‫سلينيکټومي‬
  • 6. 5 ‫ش‬‫ليدل‬ ‫ندی‬‫تسممم‬‫اعضاوو‬ ‫د‬‫يې‬‫اوسه‬‫ر‬‫ت‬‫چې‬‫ماشومان‬‫هغه‬ ‫له‬‫او‬،‫ي‬‫لر‬ ‫ژوند‬‫ښه‬‫ی‬‫و‬ ۸۰ ‫کلونو‬‫ډېرو‬‫تر‬ ‫يې‬‫ډېر‬‫څخه‬٪ .‫پايي‬ ‫پورې‬ :‫مأخذونه‬ 1. Harrison principles of internal medicine, 17th edition 2. Cecilmedicine, 23rd Edition 3. The big picture pathology, Walter l. Kemp 4. Basic Medical Biochemistry, aclinical Approach 5. Black’sMedical Dictionary, 41st edition 6. Dr.Najib’s lectures ‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬ ‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬ ‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬ ‫التوفيق‬ ‫للا‬‫من‬‫و‬ 10:50 pm/12/May/2014—‫دوشنبه‬ Kandahar, Afghanistan ‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬ ‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬‫ـ‬