| 1 | |
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| بسم الله الرحمن الرحيم بحضرتنا اليوم عن ال | |
| 2 | |
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| immunodeficiency syndrome و ال immunodeficiency | |
| 3 | |
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| syndrome are divided مرة | |
| 4 | |
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| أخرى بسم الله الرحمن الرحيم بحضرتنا اليوم عن ال | |
| 5 | |
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| immunodeficiency syndrome these are divided into | |
| 6 | |
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| two major categoriesقسمهم إلى قسمين أساسيين جاله | |
| 7 | |
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| فيه primary immune deficiency و فيه secondary | |
| 8 | |
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| immune deficiency طبعا في كلتا الحالتين في حالات | |
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| مرضية متباينة هتطرخ لأمثلة كثيرة في كل نوع من هذه | |
| 10 | |
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| الأنواع ال categories | |
| 11 | |
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| of primary immune response او deficiencyطب احنا | |
| 12 | |
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| هنتطلق في البداية لل primary immune deficiency ثم | |
| 13 | |
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| هنتقل الى ال secondary immune deficiency جانو في | |
| 14 | |
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| ال primary immune deficiency هي عبارة عن عوامل | |
| 15 | |
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| أساسية لل immune response او بالتالي بنحكي على T | |
| 16 | |
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| cell يعني cell mediated immunity و بنحكي على B | |
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| cell يعني antibody mediated immunity بنحكي على | |
| 18 | |
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| both B and T cell immunityثم Non-specific immunity | |
| 19 | |
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| متمثلة بالفاجوسيتك سيل and or انكي سيل ثم ال | |
| 20 | |
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| complement activation أيضا و طبعا كل هذه العوامل | |
| 21 | |
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| مشتركة بتمثل ال category of primary immune | |
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| response هنطلق الى كل عامل من هذه العوامل و ناخد | |
| 23 | |
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| أمثلةبداية ما كتش بتشك في وجود immunodeficiency | |
| 24 | |
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| قالوا فيه عشر محاذير أساسية مظاهر أساسية for | |
| 25 | |
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| immunodeficiency disease نمرا واحد six or more | |
| 26 | |
| 00:02:10,740 --> 00:02:14,320 | |
| infections per year يعني أكتر من ست مرات تحصل فيها | |
| 27 | |
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| infection في السماء نمرا اتنين two or more severe | |
| 28 | |
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| sinusitis per yearنمرا تلاتة antibiotic treatment | |
| 29 | |
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| lasting for two months or even longer بدون أيام | |
| 30 | |
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| visible effect نمرا أربعة two or more pneumonia | |
| 31 | |
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| per year نمرا خمسة retardation of growth and | |
| 32 | |
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| development of child نمرا ستة recurrent deep skin | |
| 33 | |
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| or organ abscessesنمرة سبعة ميكوسيس مستمر في عمل | |
| 34 | |
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| عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل | |
| 35 | |
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| عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل | |
| 36 | |
| 00:02:56,110 --> 00:02:57,110 | |
| عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل | |
| 37 | |
| 00:02:57,110 --> 00:02:57,130 | |
| عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل | |
| 38 | |
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| عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل | |
| 39 | |
| 00:02:57,530 --> 00:02:57,610 | |
| عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل | |
| 40 | |
| 00:02:57,610 --> 00:03:01,590 | |
| عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل عمل | |
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| 00:03:01,590 --> 00:03:10,320 | |
| عمل عمل عمل عمل عمل عمل عنمرا تسعة تور more severe | |
| 42 | |
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| infection زي encephalitis, osteitis, dermatitis, | |
| 43 | |
| 00:03:14,860 --> 00:03:19,440 | |
| myositis and sepsis نمرا عشرة family history | |
| 44 | |
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| indicating primary immune deficiency طبعا في عندنا | |
| 45 | |
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| جدول ان شاء الله الحلقات طلع اليه وبيبين لنا | |
| 46 | |
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| different types of infectionعلى سبيل المثال كانت | |
| 47 | |
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| بكتيريا القطاعتس ميديا و بكتيريا النومونيا غالبا | |
| 48 | |
| 00:03:41,670 --> 00:03:44,470 | |
| بنشوفها في الناس اللي عندهم B cell and antibody | |
| 49 | |
| 00:03:44,470 --> 00:03:49,970 | |
| deficiency و هكذا جدول مصنف انها بالذات أمراض | |
| 50 | |
| 00:03:49,970 --> 00:03:56,590 | |
| كثيرة و غياب كل واحد شو بيعمل فأول واحد B cell او | |
| 51 | |
| 00:03:56,590 --> 00:04:01,420 | |
| B lymphocyte deficiencywhich means deficiency in | |
| 52 | |
| 00:04:01,420 --> 00:04:05,600 | |
| antibody-mediated immunity فوق بيصير فيه recurrent | |
| 53 | |
| 00:04:05,600 --> 00:04:08,260 | |
| bacterial infection وتعتسمية recurrent infection | |
| 54 | |
| 00:04:08,260 --> 00:04:11,960 | |
| وها كذا T cell deficiency يعني cell-mediated | |
| 55 | |
| 00:04:11,960 --> 00:04:15,920 | |
| immunity deficiency بيزيد ال susceptibility to the | |
| 56 | |
| 00:04:15,920 --> 00:04:19,460 | |
| viral fungal protozoa والinfection وهي مجموعة | |
| 57 | |
| 00:04:19,460 --> 00:04:24,820 | |
| كبيرة من عيش من الحالات المرضية كل واحد له عيش له | |
| 58 | |
| 00:04:24,820 --> 00:04:27,080 | |
| ال associated disease | |
| 59 | |
| 00:04:29,920 --> 00:04:32,720 | |
| تخيل اننا نبدأ نحكي عنها ال primary | |
| 60 | |
| 00:04:32,720 --> 00:04:38,440 | |
| immunodeficiency syndromes يعني باستثناء ال IGA | |
| 61 | |
| 00:04:38,440 --> 00:04:42,260 | |
| deficiency ال frequency of immunodeficiency is | |
| 62 | |
| 00:04:42,260 --> 00:04:48,460 | |
| very low حددوها واحد في كل عشر تلاف خمسين فالمية | |
| 63 | |
| 00:04:48,460 --> 00:04:53,520 | |
| منهم associated with antibody deficiency عشرين | |
| 64 | |
| 00:04:53,520 --> 00:04:59,350 | |
| فالمية منهماسوش combined with deficiency بمعنى انه | |
| 65 | |
| 00:04:59,350 --> 00:05:03,850 | |
| فيه antibody و cell-mediated immunity 18% for | |
| 66 | |
| 00:05:03,850 --> 00:05:07,590 | |
| phagocystic disorders 10% for cell-mediated | |
| 67 | |
| 00:05:07,590 --> 00:05:15,810 | |
| immunity alone الفكرة القادمة P1.1بيبين ان كل ما | |
| 68 | |
| 00:05:15,810 --> 00:05:20,830 | |
| كان ال defect earlier كل ما كان ال genetic defect | |
| 69 | |
| 00:05:20,830 --> 00:05:24,830 | |
| او ال block حدث | |
| 70 | |
| 00:05:24,830 --> 00:05:30,330 | |
| في دراعي اللى هو الجهاز المناعى كل ما كان earlier | |
| 71 | |
| 00:05:30,330 --> 00:05:38,850 | |
| كل ما كان ال effect more severe and diseaseable | |
| 72 | |
| 00:05:38,850 --> 00:05:45,160 | |
| شوفوا الفجرطبعا هذا figure اللي انتوا شايفين يبين | |
| 73 | |
| 00:05:45,160 --> 00:05:51,460 | |
| ان ال hemopoietic maturation steps for both T and | |
| 74 | |
| 00:05:51,460 --> 00:05:58,400 | |
| B lymphocytes ف I بي على سبيل المثال بدايتها | |
| 75 | |
| 00:05:58,400 --> 00:06:04,050 | |
| lymphoid stem cellثم pre-cell ثم immature cell ثم | |
| 76 | |
| 00:06:04,050 --> 00:06:07,970 | |
| ال mature بيه lymphocytes اللي طبعا بعدها بتدينا | |
| 77 | |
| 00:06:07,970 --> 00:06:12,430 | |
| different types of antibody once اللي هو | |
| 78 | |
| 00:06:12,430 --> 00:06:18,090 | |
| maturation occur to plasma cell في كل مرحلة من | |
| 79 | |
| 00:06:18,090 --> 00:06:23,570 | |
| المراحل ممكن يحصل فيه defect وكل مرحلة من المراحل | |
| 80 | |
| 00:06:23,570 --> 00:06:27,950 | |
| associated with certain diseaseو كل ما كانت | |
| 81 | |
| 00:06:27,950 --> 00:06:36,070 | |
| المرحلة أو الخلل بدري كل ما كانش كل المراحل التي | |
| 82 | |
| 00:06:36,070 --> 00:06:41,710 | |
| تلي الخلل بدها تتعطل وبالتالي كل ما كان ال disease | |
| 83 | |
| 00:06:41,710 --> 00:06:45,290 | |
| more severe نفس الحكاية بالنسبة لل thymus او ال | |
| 84 | |
| 00:06:45,290 --> 00:06:49,750 | |
| telephocytes هي بتيجي على ال thymus | |
| 85 | |
| 00:06:52,580 --> 00:06:59,000 | |
| بعد ذلك تظهر كـ mature CD4 أو CD8 T lymphocytes | |
| 86 | |
| 00:06:59,000 --> 00:07:05,280 | |
| وطبعا تشتغل كimmune | |
| 87 | |
| 00:07:05,280 --> 00:07:10,600 | |
| cell المهم انتبهوا إلى الأرقام الموجودة فشوفوا | |
| 88 | |
| 00:07:10,600 --> 00:07:15,600 | |
| قسموا المراحل هذه إلى أرقام مختلفة وكل مرحلة | |
| 89 | |
| 00:07:15,600 --> 00:07:19,120 | |
| associated زي ما قلتلكم or combined with a certain | |
| 90 | |
| 00:07:19,120 --> 00:07:24,000 | |
| disease فرق 1 على سبيل المثال هييعني فى بدري خالص | |
| 91 | |
| 00:07:24,000 --> 00:07:27,120 | |
| الخلال بيصير فى severe compound immunodeficiency | |
| 92 | |
| 00:07:27,120 --> 00:07:32,200 | |
| syndrome رقم اتنين برضه بدري ادوة بيصير فى | |
| 93 | |
| 00:07:32,200 --> 00:07:36,000 | |
| congenital thymic aplasia وبرضه هذا عبارة عن دي | |
| 94 | |
| 00:07:36,000 --> 00:07:40,020 | |
| جارس سندروم يعنى مافيش تى lymphocytes بتتصنع اتول | |
| 95 | |
| 00:07:40,020 --> 00:07:47,480 | |
| رقم تلاتة ادوة بيصير فى skid ايضا رقم اربع فى ال B | |
| 96 | |
| 00:07:47,480 --> 00:07:51,610 | |
| cell ادوةقالوا بيصير في X-Linked E Gamma Global | |
| 97 | |
| 00:07:51,610 --> 00:07:58,530 | |
| Anemia مافي ولا Antibody بيتكون رقم خمسة ماشي هذا | |
| 98 | |
| 00:07:58,530 --> 00:08:03,570 | |
| هو أنا رقم خمسة بيصير common variable immune | |
| 99 | |
| 00:08:03,570 --> 00:08:09,670 | |
| deficiency disease و هكذا كل الحالات اللي هو كل | |
| 100 | |
| 00:08:09,670 --> 00:08:16,440 | |
| المراحل associated with certain diseaseنبدأ بـ | |
| 101 | |
| 00:08:16,440 --> 00:08:22,600 | |
| Severe Combined Immunodeficiency Diseases وهي | |
| 102 | |
| 00:08:22,600 --> 00:08:26,640 | |
| عبارة عن هيتروجينس جروب of disease associated or | |
| 103 | |
| 00:08:26,640 --> 00:08:29,700 | |
| in which cell-mediated immunity and antibody لأنه | |
| 104 | |
| 00:08:29,700 --> 00:08:35,060 | |
| combined production are defected التانين معطلين | |
| 105 | |
| 00:08:35,060 --> 00:08:38,960 | |
| both humoral and cell-mediated immunity are | |
| 106 | |
| 00:08:38,960 --> 00:08:44,940 | |
| defectedفي الزمن هتسموها او Swiss-type Aiga | |
| 107 | |
| 00:08:44,940 --> 00:08:49,820 | |
| Microbial Anemia و الناس هدولة عادة susceptible to | |
| 108 | |
| 00:08:49,820 --> 00:08:54,060 | |
| virtually every type of microbial infection, viral | |
| 109 | |
| 00:08:54,060 --> 00:08:59,040 | |
| infection, bacterial infection, fungal, protozoal | |
| 110 | |
| 00:08:59,040 --> 00:09:04,380 | |
| and so on أشهر الحاجات اللي ملحوظة في هذا النوع من | |
| 111 | |
| 00:09:04,380 --> 00:09:08,380 | |
| الأمراض هو Infection of Cytomegalovirus, | |
| 112 | |
| 00:09:08,980 --> 00:09:16,220 | |
| Pneumocystitiscarny eye and candida طبعا الناس دول | |
| 113 | |
| 00:09:16,220 --> 00:09:23,260 | |
| ممنوع ياخدوا vaccine with attenuated lymph virus | |
| 114 | |
| 00:09:23,260 --> 00:09:29,700 | |
| لأنه it will lead to death patient have been | |
| 115 | |
| 00:09:29,700 --> 00:09:32,260 | |
| classified according to the following figure or | |
| 116 | |
| 00:09:32,260 --> 00:09:40,020 | |
| table جال ودوا قسمهم ل T minus B plus T minus B | |
| 117 | |
| 00:09:40,020 --> 00:09:45,920 | |
| minusأنواع مختلفة سنتطرق لكل نوع من هذه الأنواع بس | |
| 118 | |
| 00:09:45,920 --> 00:09:51,980 | |
| يجب أن نفهم ان T minus يعني في غياب ل T cell بي | |
| 119 | |
| 00:09:51,980 --> 00:09:57,500 | |
| بلس يعني في normal بي cell وممكن يكون في غياب | |
| 120 | |
| 00:09:57,500 --> 00:10:02,280 | |
| لأنكي cell لما أقول T minus بي minus يعني لا بي | |
| 121 | |
| 00:10:02,280 --> 00:10:08,780 | |
| ولا T cell موجودة و لما أقول التنين plusمعناته | |
| 122 | |
| 00:10:08,780 --> 00:10:12,080 | |
| الناس التانية هدول عندهم T سل وB سل لكن هم غير | |
| 123 | |
| 00:10:12,080 --> 00:10:18,840 | |
| شغالاء مش شغالين T plus B minus it's very rare و | |
| 124 | |
| 00:10:18,840 --> 00:10:23,960 | |
| they preferred treatment طبعا لحالات من هالنوع انه | |
| 125 | |
| 00:10:23,960 --> 00:10:28,460 | |
| نعمل bone marrow transplantation from an HLA | |
| 126 | |
| 00:10:28,460 --> 00:10:33,660 | |
| matched sibling donor وده بقى و هذه الحالات اول | |
| 127 | |
| 00:10:33,660 --> 00:10:38,340 | |
| حالة هي T minus B plus اتفقنا على انه هيمافيش D | |
| 128 | |
| 00:10:38,340 --> 00:10:42,000 | |
| -cell وإنما مافيه B-cell المرض هذا عبارة عن X | |
| 129 | |
| 00:10:42,000 --> 00:10:49,920 | |
| -linked skid بيمثل 40 ل 50% من الحالات اللي بنحكي | |
| 130 | |
| 00:10:49,920 --> 00:10:54,000 | |
| عنها ال mutation جاله موجود على ال X-chromosome | |
| 131 | |
| 00:10:54,000 --> 00:10:58,980 | |
| الجين اللي صار فيه mutation موجود على ال X | |
| 132 | |
| 00:10:58,980 --> 00:11:05,180 | |
| -chromosome which could forgamer chain ماشي | |
| 133 | |
| 00:11:06,650 --> 00:11:12,870 | |
| Gamma Chain Cytokine Receptor وطبعاً this Gamma | |
| 134 | |
| 00:11:12,870 --> 00:11:17,230 | |
| Chain تلعب دور كبير في ال .. اللي هو Signal | |
| 135 | |
| 00:11:17,230 --> 00:11:22,290 | |
| Transduction لعدد كبير من ال cytokine منها IL2, | |
| 136 | |
| 00:11:22,390 --> 00:11:30,110 | |
| IL4, IL7, IL9, IL15وطبعا هذه معناته غيبها بعمل | |
| 137 | |
| 00:11:30,110 --> 00:11:36,930 | |
| غياب ل ال function for this cytokine وهذا فكرة | |
| 138 | |
| 00:11:36,930 --> 00:11:42,830 | |
| واضح جدا هي receptor اللي هو عبارة عن إيش legend | |
| 139 | |
| 00:11:42,830 --> 00:11:49,870 | |
| لكل ال cytokine هذول ال legend تعونوه لكل ال | |
| 140 | |
| 00:11:49,870 --> 00:11:55,690 | |
| cytokine هذول فهي بيتكوم من two chain واحدةتو بولي | |
| 141 | |
| 00:11:55,690 --> 00:11:58,550 | |
| مبتعدة chain واحدة منهم بتلعب دوري كبير في ال | |
| 142 | |
| 00:11:58,550 --> 00:12:03,010 | |
| signal transduction وهي ال gamma chain gamma chain | |
| 143 | |
| 00:12:03,010 --> 00:12:05,690 | |
| وبالتالي غيابها يعني مافيش signal transduction | |
| 144 | |
| 00:12:05,690 --> 00:12:10,270 | |
| وبالتالي مافيش transcription for this gene ماشي | |
| 145 | |
| 00:12:10,270 --> 00:12:16,330 | |
| وبالتالي كل ال function لهذه ال cytokine will be | |
| 146 | |
| 00:12:16,330 --> 00:12:22,950 | |
| missed المرض | |
| 147 | |
| 00:12:22,950 --> 00:12:30,260 | |
| التاني ال witchesيعني similar to this disease هو | |
| 148 | |
| 00:12:30,260 --> 00:12:34,880 | |
| غياب الجزء التاني من ال receptor مش كبوليبين بتعدد | |
| 149 | |
| 00:12:34,880 --> 00:12:39,000 | |
| شينو انما ك functioning process اللي هو غياب الجاك | |
| 150 | |
| 00:12:39,000 --> 00:12:44,900 | |
| يوم ما يصير فيه غياب للجاك طبعا ما يصيرش فيه تغير | |
| 151 | |
| 00:12:44,900 --> 00:12:47,440 | |
| في ال gamma ومتما تغيرت بتاعة ال signal | |
| 152 | |
| 00:12:47,440 --> 00:12:52,190 | |
| transduction غياب الجاك أيضابرضه بعمل نفس الأعضاء | |
| 153 | |
| 00:12:52,190 --> 00:12:57,950 | |
| وبالتالي بيصير فيه غياب لل signal transduction هذا | |
| 154 | |
| 00:12:57,950 --> 00:13:04,990 | |
| X-linked وال Jack deficiency هو عبارة عن اتزوم ال | |
| 155 | |
| 00:13:04,990 --> 00:13:12,290 | |
| recessive disease اي | |
| 156 | |
| 00:13:12,290 --> 00:13:15,670 | |
| اتزوم ال recessive قلنا غياب ال Jack تلاتة هو | |
| 157 | |
| 00:13:15,670 --> 00:13:21,290 | |
| عبارة عن Tyrosine Kinase وطبعا غياببيعني مافيش | |
| 158 | |
| 00:13:21,290 --> 00:13:24,950 | |
| signal transduction ووجدوا ان ال expression of | |
| 159 | |
| 00:13:24,950 --> 00:13:28,530 | |
| Jack موجود في كل ال hemopoietic cells وبالتالي | |
| 160 | |
| 00:13:28,530 --> 00:13:33,790 | |
| مايصيرش في maturation للخلايا | |
| 161 | |
| 00:13:33,790 --> 00:13:40,310 | |
| هذه النقطة التانية T minus B minus ومن الأمراض | |
| 162 | |
| 00:13:40,310 --> 00:13:46,830 | |
| المشاركة في هذا ال group هو ال adenosine diamine | |
| 163 | |
| 00:13:46,830 --> 00:13:52,160 | |
| deficiencyوهذا عبارة عن نزاين بيلعب دور كبير في | |
| 164 | |
| 00:13:52,160 --> 00:13:59,660 | |
| الـ Purine pathway يمثل 20% من حالات الـ Skid وهو | |
| 165 | |
| 00:13:59,660 --> 00:14:05,260 | |
| عبارة عن autism recessive disease وغيابه بيعني | |
| 166 | |
| 00:14:05,260 --> 00:14:11,720 | |
| accumulation of toxic wastes بيصير في build up في | |
| 167 | |
| 00:14:11,720 --> 00:14:20,200 | |
| build up ليش لل toxic waste في الجسمطبعا it has | |
| 168 | |
| 00:14:20,200 --> 00:14:24,340 | |
| greatest impact of the immune على الجهاز المناعى | |
| 169 | |
| 00:14:24,340 --> 00:14:29,880 | |
| بيأدى للفيلير أو both T and B cell development بعد | |
| 170 | |
| 00:14:29,880 --> 00:14:32,260 | |
| تانى اللى هو purely nucleotide phosphorylase | |
| 171 | |
| 00:14:32,260 --> 00:14:33,020 | |
| deficiency | |
| 172 | |
| 00:14:35,470 --> 00:14:39,610 | |
| هذا سيؤدي أيضًا إلى بناء تجارب من أجهزة مخاطرة | |
| 173 | |
| 00:14:39,610 --> 00:14:47,910 | |
| خصوصًا هذه الأجهزة المخاطرة بإمكانها تدمر نظام | |
| 174 | |
| 00:14:47,910 --> 00:14:52,930 | |
| الهيولوجيا وبيأثر | |
| 175 | |
| 00:14:52,930 --> 00:14:57,570 | |
| أيضًا على الـ T-cell وبالتالي كل الانفويتشيو | |
| 176 | |
| 00:14:57,570 --> 00:15:03,470 | |
| مدفنين نوع تاني اللي هو Recombinase deficiency | |
| 177 | |
| 00:15:04,630 --> 00:15:09,150 | |
| أيضاً الـ Recombinase Deficiency نعرف أنه في | |
| 178 | |
| 00:15:09,150 --> 00:15:13,610 | |
| الجينات المسؤولة اللي هي RAG 1 و RAG 2 هما two | |
| 179 | |
| 00:15:13,610 --> 00:15:16,750 | |
| important genes موجودين فى الـ lymphocytes only | |
| 180 | |
| 00:15:16,750 --> 00:15:21,710 | |
| وبيلعبوا دور كبير فى ال rearrangement of both | |
| 181 | |
| 00:15:21,710 --> 00:15:28,950 | |
| immunoglobulin gene and TCR gene وبالتالي غيابهم | |
| 182 | |
| 00:15:29,520 --> 00:15:34,520 | |
| بيؤدي إلى خلل في الـ gene rearrangement وبالتالي | |
| 183 | |
| 00:15:34,520 --> 00:15:38,600 | |
| الـ mutation بتؤثر إلى complete absence تعمل | |
| 184 | |
| 00:15:38,600 --> 00:15:43,440 | |
| complete absence للـ T و B الانفسايت وبالتالي | |
| 185 | |
| 00:15:43,440 --> 00:15:44,540 | |
| مافيش immunoglobul | |
| 186 | |
| 00:15:50,790 --> 00:15:56,210 | |
| وبالتالي مافيش match or لأ تي ولا بي لأن ال | |
| 187 | |
| 00:15:56,210 --> 00:16:04,670 | |
| function is intact تي بلس بي ماعين اذا بيقولوا | |
| 188 | |
| 00:16:06,040 --> 00:16:10,740 | |
| وقالوا ان أشارها الـ Omen Syndrome والـ Omen | |
| 189 | |
| 00:16:10,740 --> 00:16:17,280 | |
| Syndrome أيضًا هو عبارة عن خلل بيصير فيه reduction | |
| 190 | |
| 00:16:17,280 --> 00:16:21,420 | |
| في ال or partial complete or partial reduction of | |
| 191 | |
| 00:16:21,420 --> 00:16:27,900 | |
| the rag genes وبالتالي بيصير فيه خلل في ال rag | |
| 192 | |
| 00:16:27,900 --> 00:16:32,760 | |
| activity ال clinical presentation زي وزي ال graft | |
| 193 | |
| 00:16:32,760 --> 00:16:39,350 | |
| versus host diseaseوالناس دولة تدفع هذه التحكمات | |
| 194 | |
| 00:16:39,350 --> 00:16:44,390 | |
| للنظام الانيونيو وهم لديهم جسد كبير جي تي | |
| 195 | |
| 00:16:44,390 --> 00:16:51,550 | |
| انفلتراتيشن من زينوفيل واكتفيت تي سيل تدفعنا ال | |
| 196 | |
| 00:16:51,550 --> 00:16:57,290 | |
| تيهيلبرتو باتوي سايتو كاين وهذا بيقدر الهيبر اي جي | |
| 197 | |
| 00:16:57,290 --> 00:17:00,730 | |
| اي سيندروم ومال نيوتراشين | |
| 198 | |
| 00:17:03,450 --> 00:17:08,590 | |
| المحاصلة مرضى من هذا النوع بيحتاج الى pre | |
| 199 | |
| 00:17:08,590 --> 00:17:14,430 | |
| -treatment with immunosuppressive drug ال | |
| 200 | |
| 00:17:14,430 --> 00:17:21,550 | |
| T plus B plus subgroup كل التنتين موجودين لكن they | |
| 201 | |
| 00:17:21,550 --> 00:17:24,870 | |
| are not functioning أشهر هذه الأمراض ال pair | |
| 202 | |
| 00:17:24,870 --> 00:17:30,040 | |
| lymphocyte syndromeده هو بيقدي الى results from | |
| 203 | |
| 00:17:30,040 --> 00:17:34,920 | |
| the failure to express HLA molecule والسبب في ذلك | |
| 204 | |
| 00:17:34,920 --> 00:17:37,960 | |
| طبعا مافيش HLA molecule يعني مافيش antigen | |
| 205 | |
| 00:17:37,960 --> 00:17:45,220 | |
| presentation بواسطة طبعا اللي هو HLA class 2 | |
| 206 | |
| 00:17:45,220 --> 00:17:48,680 | |
| molecule deficiency في الغالب class 1 ممكن يكون | |
| 207 | |
| 00:17:48,680 --> 00:17:54,470 | |
| متأثر وممكن لأcirculating T and B cell may be | |
| 208 | |
| 00:17:54,470 --> 00:18:00,110 | |
| normal لكن في غياب ال HLA class 2 موليكيول ما | |
| 209 | |
| 00:18:00,110 --> 00:18:07,180 | |
| بيحصل فيه antigen presentation ل CD4 T cellالـ ZAP | |
| 210 | |
| 00:18:07,180 --> 00:18:12,080 | |
| -17 ميوتيشن هي عبارة عن كيميكال | |
| 211 | |
| 00:18:12,080 --> 00:18:19,620 | |
| ميسنجر بيعمل نقل للإشارة بمنخد | |
| 212 | |
| 00:18:19,620 --> 00:18:21,700 | |
| TCR للنوكلياس وهو نقل للإشارة بمنخد TCR للنوكلياس | |
| 213 | |
| 00:18:21,700 --> 00:18:24,800 | |
| وهو نقل للإشارة بمنخد TCR للنوكلياس وهو نقل | |
| 214 | |
| 00:18:24,800 --> 00:18:27,880 | |
| للإشارة | |
| 215 | |
| 00:18:27,880 --> 00:18:31,840 | |
| بمنخد TCR للنوكلياسشوفوا هذه العلاقة ما أحلاها | |
| 216 | |
| 00:18:31,840 --> 00:18:37,580 | |
| نعرف انه في عندنا antigen presenting cell ماشي | |
| 217 | |
| 00:18:37,580 --> 00:18:43,160 | |
| بتقدم اللي هو | |
| 218 | |
| 00:18:43,160 --> 00:18:47,400 | |
| telophosize | |
| 219 | |
| 00:18:47,400 --> 00:18:56,040 | |
| ال antigen ل telophosize و طبعاسواء كانت الـ | |
| 220 | |
| 00:18:56,040 --> 00:19:01,140 | |
| Antigen Presenting Cell Macrophage او B Cell تنتهي | |
| 221 | |
| 00:19:01,140 --> 00:19:05,660 | |
| بقدم الانتجين لمن؟ لـ T-Lymphocyte شوفوا احتمالات | |
| 222 | |
| 00:19:05,660 --> 00:19:09,340 | |
| الخلال كيف بتصير قبل احتمال يصير فيه خلال في ال | |
| 223 | |
| 00:19:09,340 --> 00:19:13,220 | |
| MSC Class II بيعمل مرض يسمونه Pale Lymphocyte | |
| 224 | |
| 00:19:13,220 --> 00:19:16,740 | |
| Syndrome حاجة نقال متوم احتمال الخلاز يكون في ال | |
| 225 | |
| 00:19:16,740 --> 00:19:20,860 | |
| FAS او ال FAS Legend و هيعمل FAS Deficiency | |
| 226 | |
| 00:19:20,860 --> 00:19:27,110 | |
| Syndromeو احتمال الخلق يكون في CD40 legend و CD40 | |
| 227 | |
| 00:19:27,110 --> 00:19:30,590 | |
| legend موجود على ال T cell بيعمل حاجة بيسموها | |
| 228 | |
| 00:19:30,590 --> 00:19:37,990 | |
| Hyper IgM Syndrome اثر | |
| 229 | |
| 00:19:37,990 --> 00:19:43,150 | |
| Multi System Disorders بالاضافة لل skid في skid | |
| 230 | |
| 00:19:43,150 --> 00:19:48,190 | |
| like clinical picture associated with certain | |
| 231 | |
| 00:19:48,190 --> 00:19:52,000 | |
| disease زي Stuttgart-Aldrich Syndromeودا اسمه | |
| 232 | |
| 00:19:52,000 --> 00:19:56,940 | |
| syndrome عبارة عن x-linked disease بيتمثل بالقاتل | |
| 233 | |
| 00:19:56,940 --> 00:20:00,860 | |
| نمرا واحد في bleeding لأنه فيه thrombocytopenia | |
| 234 | |
| 00:20:00,860 --> 00:20:04,340 | |
| نمرا اتنين في recurrent bacterial infection في | |
| 235 | |
| 00:20:04,340 --> 00:20:12,200 | |
| allergic reaction في long term patient ممكن يجلب ل | |
| 236 | |
| 00:20:12,200 --> 00:20:18,680 | |
| malignant disease و ال genetic basisنت يعني | |
| 237 | |
| 00:20:18,680 --> 00:20:24,580 | |
| ميوتيشن في الـ X-linked gene اللي بيقدّي الى a | |
| 238 | |
| 00:20:24,580 --> 00:20:29,940 | |
| witch code for ويسكوت ألدريخ سندروم بروتين وهذا | |
| 239 | |
| 00:20:29,940 --> 00:20:35,400 | |
| موجود في كل الـ hemopoietic stem cells both T and | |
| 240 | |
| 00:20:35,400 --> 00:20:38,220 | |
| B cell are functionally non abnormal | |
| 241 | |
| 00:20:40,700 --> 00:20:47,340 | |
| وبالتالي لا يمكن الشخص ان يتفاعل بـ Polysaccharide | |
| 242 | |
| 00:20:47,340 --> 00:20:51,640 | |
| Antigen التعامل في هذه الحالة يحتاج إلى Antibiotic | |
| 243 | |
| 00:20:51,640 --> 00:20:57,600 | |
| وAntiviral و بولمارو ترانسبلانتيشن و بدون هذا | |
| 244 | |
| 00:20:57,600 --> 00:21:02,260 | |
| العلاج ال life expectancy لا تتعدى تلت سنوات | |
| 245 | |
| 00:21:02,260 --> 00:21:09,180 | |
| أتاكسيا تلانجكتازيامن القصير برضه مرضى system | |
| 246 | |
| 00:21:09,180 --> 00:21:14,320 | |
| genetic disorders بيتمثل بالقاتل بيصير فيه jerky | |
| 247 | |
| 00:21:14,320 --> 00:21:19,480 | |
| movement or lack of muscle coordination ونمر اتنين | |
| 248 | |
| 00:21:19,480 --> 00:21:24,170 | |
| abnormal vascular dilatation تلعنجكتازيةفي الـ eye | |
| 249 | |
| 00:21:24,170 --> 00:21:28,570 | |
| و ال skin و بزيادة ال susceptibility to infection | |
| 250 | |
| 00:21:28,570 --> 00:21:34,870 | |
| بيصير اندولنفومة و ال defect في ال both B و T cell | |
| 251 | |
| 00:21:34,870 --> 00:21:40,270 | |
| ال gene طبعا of ataxia telangiectasia code for a | |
| 252 | |
| 00:21:40,270 --> 00:21:46,490 | |
| protein known ATM الأطفال have a risk for | |
| 253 | |
| 00:21:46,490 --> 00:21:51,870 | |
| developing malignancy خصوصا ال lymphoid neoplasm | |
| 254 | |
| 00:21:55,810 --> 00:22:01,390 | |
| الـ category التاني هو ال immune deficiency | |
| 255 | |
| 00:22:01,390 --> 00:22:04,030 | |
| disorder associated with T cell and cell-mediated | |
| 256 | |
| 00:22:04,030 --> 00:22:08,510 | |
| immunity قالوا الناس هدولة مش عندهم T cell | |
| 257 | |
| 00:22:08,510 --> 00:22:12,790 | |
| associated deficiency disease انه اتفقنا على ان ال | |
| 258 | |
| 00:22:12,790 --> 00:22:19,090 | |
| T cell deficiency ماهيشي associated with اللي هو | |
| 259 | |
| 00:22:19,090 --> 00:22:21,810 | |
| disease زي ال viral fungal protozoal infection | |
| 260 | |
| 00:22:23,590 --> 00:22:26,890 | |
| وكلنا بنعرف إن الـ T-Lymphocyte بتساعد الـ B | |
| 261 | |
| 00:22:26,890 --> 00:22:30,730 | |
| -Lymphocyte في إنتاج الـ antibody which antibody | |
| 262 | |
| 00:22:30,730 --> 00:22:36,430 | |
| would form T cell dependent antigen وغياب or T | |
| 263 | |
| 00:22:36,430 --> 00:22:40,810 | |
| cell associated deficiency هيعمل selective defect | |
| 264 | |
| 00:22:40,810 --> 00:22:46,170 | |
| in antibody production وبالتالي T cell deficient | |
| 265 | |
| 00:22:46,170 --> 00:22:51,430 | |
| patient have difficultto distinguish have | |
| 266 | |
| 00:22:51,430 --> 00:22:55,850 | |
| difficulty to distinguish clinically to be | |
| 267 | |
| 00:22:55,850 --> 00:23:00,270 | |
| distinguished clinically from a skeptic patient ما | |
| 268 | |
| 00:23:00,270 --> 00:23:05,450 | |
| زال ال B و ال T مش موجودة يبقى كمان ال B مش راح | |
| 269 | |
| 00:23:05,450 --> 00:23:09,350 | |
| تشتغل Congenital | |
| 270 | |
| 00:23:09,350 --> 00:23:13,890 | |
| Thymic Aplasia اللي هو ديجارد سندروم قلنا هذا في | |
| 271 | |
| 00:23:13,890 --> 00:23:20,950 | |
| مراحل مبكرة جدا بصير اذا فاكرين في رقم اتنينفي الـ | |
| 272 | |
| 00:23:20,950 --> 00:23:26,110 | |
| T cell hemopoietic maturation steps المهم دي جورج | |
| 273 | |
| 00:23:26,110 --> 00:23:31,070 | |
| سندروم بيعني انه فيه T cell deficiency و in which | |
| 274 | |
| 00:23:31,070 --> 00:23:34,770 | |
| the cell must develop abnormally مافيش ايش مافيش | |
| 275 | |
| 00:23:34,770 --> 00:23:38,950 | |
| development يبقى مافي إنتاج ل T lymphocyte no T | |
| 276 | |
| 00:23:38,950 --> 00:23:46,310 | |
| cell و نقول إن الخلل ناتج عن defective migration | |
| 277 | |
| 00:23:46,310 --> 00:23:51,420 | |
| of fetal neural crest cellsالثالثة والتالتة | |
| 278 | |
| 00:23:51,420 --> 00:23:58,280 | |
| مصطلحات الفرنجيال وبالتالي المريض الهارد والفيس | |
| 279 | |
| 00:23:58,280 --> 00:24:05,480 | |
| يتطور بشكل طبيعي وبرضه الثايمس والبراثيرويد لا يتم | |
| 280 | |
| 00:24:05,480 --> 00:24:08,820 | |
| تصميم هذا يؤدي إلى ثايمك أبليجيا | |
| 281 | |
| 00:24:08,820 --> 00:24:14,640 | |
| وhypoparathyroidism ثايمك | |
| 282 | |
| 00:24:14,640 --> 00:24:18,940 | |
| أبليجيا ينتهي في مفاجأة مدرسة مدرسة مدرسة مدرسة | |
| 283 | |
| 00:24:19,890 --> 00:24:24,170 | |
| Dudler's syndrome هو مش hereditary disease وانما | |
| 284 | |
| 00:24:24,170 --> 00:24:31,990 | |
| بيصير فيه Sporadic deficiency in chromosome 22q11 | |
| 285 | |
| 00:24:31,990 --> 00:24:36,990 | |
| or deletion طبعا هدول المرضى بيصير في عندهم | |
| 286 | |
| 00:24:36,990 --> 00:24:40,350 | |
| recurrent of or chronic infection with viruses | |
| 287 | |
| 00:24:40,350 --> 00:24:47,810 | |
| بكتيريا فنجاى بروتوزوهو و الناس هدول عندهم قياب لل | |
| 288 | |
| 00:24:47,810 --> 00:24:54,090 | |
| T cellأيضًا فشلت تجري على تجارب الانتباه هذه | |
| 289 | |
| 00:24:54,090 --> 00:24:56,730 | |
| الأشخاص لا يجب أن يكونوا مميزين بواسطة مواجهة | |
| 290 | |
| 00:24:56,730 --> 00:24:58,610 | |
| مواجهة مواجهة مواجهة مواجهة مواجهة مواجهة مواجهة | |
| 291 | |
| 00:24:58,610 --> 00:25:02,910 | |
| مواجهة مواجهة مواجهة | |
| 292 | |
| 00:25:03,160 --> 00:25:06,740 | |
| with normal peripheral T cell number يعني فيه | |
| 293 | |
| 00:25:06,740 --> 00:25:12,140 | |
| normal T lymphocyte لكن في نفس الوقت فشي فشي | |
| 294 | |
| 00:25:12,140 --> 00:25:17,960 | |
| function ادى functional defect فى T cell ايضا | |
| 295 | |
| 00:25:17,960 --> 00:25:22,260 | |
| opportunistic infection and high incidence of | |
| 296 | |
| 00:25:22,260 --> 00:25:27,140 | |
| autoimmune disease ملاحظات it's autosomal | |
| 297 | |
| 00:25:27,140 --> 00:25:32,200 | |
| recessive pattern وطبعا هو عبارة عن heterogeneous | |
| 298 | |
| 00:25:32,200 --> 00:25:38,880 | |
| groupأيضا يشمل غياب لل ZAP-17 تيروزينكينيز CD3E | |
| 299 | |
| 00:25:38,880 --> 00:25:45,860 | |
| وCD3G هو هذه الموليكيول المتعلقة بالإشعار T-cell | |
| 300 | |
| 00:25:45,860 --> 00:25:55,840 | |
| وبالتالي مافيش فانكشف Autoimmune | |
| 301 | |
| 00:25:55,840 --> 00:26:01,920 | |
| lymphoproliferative disease اوأيضاً إنها تسمى بـ | |
| 302 | |
| 00:26:01,920 --> 00:26:06,300 | |
| Inherited by Massive Proliferation of Lymphoid | |
| 303 | |
| 00:26:06,300 --> 00:26:08,980 | |
| Tissue وبدأت | |
| 304 | |
| 00:26:08,980 --> 00:26:27,160 | |
| يتطططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططططط | |
| 305 | |
| 00:26:32,150 --> 00:26:35,050 | |
| عامل الـ autoimmune lymphoproliferative الشخصية أو | |
| 306 | |
| 00:26:35,050 --> 00:26:43,170 | |
| الشخصية السينامية لديها ميوتيشن في الـ FAS جين | |
| 307 | |
| 00:26:43,170 --> 00:26:51,870 | |
| يتعامل مع الـ FAS بروتين CD95 يتعامل مع الـ | |
| 308 | |
| 00:26:51,870 --> 00:27:00,350 | |
| Apoptosis signaling لديهم واحد طبيعي وواحد ميوتيت | |
| 309 | |
| 00:27:00,350 --> 00:27:07,140 | |
| FAS موليكولوهذا بيؤدي او that mutated phase | |
| 310 | |
| 00:27:07,140 --> 00:27:11,140 | |
| molecule interfere with the function of normal | |
| 311 | |
| 00:27:11,140 --> 00:27:15,780 | |
| molecule طبعا ال mutated هو اللي بيتدخل في ال | |
| 312 | |
| 00:27:15,780 --> 00:27:20,420 | |
| function of normal molecule these patients have | |
| 313 | |
| 00:27:20,420 --> 00:27:25,840 | |
| defect in other component of apoptosis pathway زي | |
| 314 | |
| 00:27:25,840 --> 00:27:29,120 | |
| ال phase ligand or case base 10 | |
| 315 | |
| 00:27:31,670 --> 00:27:37,270 | |
| Chronic Mucocutaneous Candidiasis بيه من الاسم | |
| 316 | |
| 00:27:37,270 --> 00:27:42,250 | |
| Candidiasis يعني فيه Candida infection of skin and | |
| 317 | |
| 00:27:42,250 --> 00:27:47,830 | |
| mucous membrane غالبا المرضى have normal T cell | |
| 318 | |
| 00:27:47,830 --> 00:27:52,070 | |
| mediated immunity to microorganism other than | |
| 319 | |
| 00:27:52,070 --> 00:27:56,810 | |
| Candida and normal B cell mediated immunityto all | |
| 320 | |
| 00:27:56,810 --> 00:28:04,190 | |
| microorganism بما فيها ال Canada يعني الخلل في | |
| 321 | |
| 00:28:04,190 --> 00:28:09,210 | |
| ايه؟ في ال T-lymphocyte which can respond to all | |
| 322 | |
| 00:28:09,210 --> 00:28:12,930 | |
| ال micro part مع ال Canada بينما ال B-lymphocyte | |
| 323 | |
| 00:28:12,930 --> 00:28:17,350 | |
| is normal they have selective defect in the | |
| 324 | |
| 00:28:17,350 --> 00:28:20,210 | |
| functional of T cell | |
| 325 | |
| 00:28:23,500 --> 00:28:28,520 | |
| التاني هو ال BCL-R immunoglobulin associated | |
| 326 | |
| 00:28:28,520 --> 00:28:33,400 | |
| immunodeficiency وأشهر | |
| 327 | |
| 00:28:33,400 --> 00:28:36,980 | |
| مثال على ذلك بروتون اي جاما غلوبيلانينيا وهو عبارة | |
| 328 | |
| 00:28:36,980 --> 00:28:42,340 | |
| عن X-linked infantile اي جاما غلوبيلانينيا الأطفال | |
| 329 | |
| 00:28:42,340 --> 00:28:48,260 | |
| من Infanta الأطفال present with serious unrepeated | |
| 330 | |
| 00:28:48,260 --> 00:28:52,390 | |
| infectionوسبب في ذلك is severe depression or | |
| 331 | |
| 00:28:52,390 --> 00:28:56,710 | |
| virtual absence of all immunoglobulin نحنا بنحكي | |
| 332 | |
| 00:28:56,710 --> 00:29:00,890 | |
| عليها protons يعني مافيش بي سل مافيش ايش بي | |
| 333 | |
| 00:29:00,890 --> 00:29:06,650 | |
| lymphocyte معناته مافيش antibody production طبعا | |
| 334 | |
| 00:29:06,650 --> 00:29:11,250 | |
| ال major defect وجدوه انه في عدم قدرة البيت انها | |
| 335 | |
| 00:29:11,250 --> 00:29:19,290 | |
| تجلب to be mature as B cell protons جين | |
| 336 | |
| 00:29:20,990 --> 00:29:28,470 | |
| تم تغييره بطريقة X مرتبطة هذه | |
| 337 | |
| 00:29:28,470 --> 00:29:33,710 | |
| الجيوانات تدعى تيروزين كينيز انزعيم موجود في | |
| 338 | |
| 00:29:33,710 --> 00:29:39,150 | |
| الـCytosol XLA | |
| 339 | |
| 00:29:39,150 --> 00:29:44,810 | |
| تم تحيطه لعقل عقلي المتوسط المتوسط | |
| 340 | |
| 00:29:44,810 --> 00:29:46,210 | |
| الموجود في الـCytoplasmic | |
| 341 | |
| 00:29:49,190 --> 00:29:53,930 | |
| Patient suffered repeated infection Treatment طبعا | |
| 342 | |
| 00:29:53,930 --> 00:29:57,490 | |
| Periodic injection of intravenous gamma globulin | |
| 343 | |
| 00:29:57,490 --> 00:30:04,670 | |
| بيحتوي على IgG Transient | |
| 344 | |
| 00:30:04,670 --> 00:30:10,990 | |
| Hypo Gamma Globulinemia اسم Transient في عمر خمسة | |
| 345 | |
| 00:30:10,990 --> 00:30:16,940 | |
| لست شهوربيصير فيه Passively transferred from | |
| 346 | |
| 00:30:16,940 --> 00:30:26,980 | |
| maternal to fetus of IgG antibody وطبعا | |
| 347 | |
| 00:30:26,980 --> 00:30:36,140 | |
| في هذه الحالة بيبدأ ال IGG بيبدأ يظهر | |
| 348 | |
| 00:30:36,140 --> 00:30:44,810 | |
| اللي هو ال infant IGG premature infantممكن يصير | |
| 349 | |
| 00:30:44,810 --> 00:30:49,570 | |
| عنده Transient IgG Deficiency if they are not yet | |
| 350 | |
| 00:30:49,570 --> 00:30:55,050 | |
| able to synthesize immunoglobulin sometimes helper | |
| 351 | |
| 00:30:55,050 --> 00:30:59,630 | |
| T cells are deficient يعني بيصير فيه غياب في ال T | |
| 352 | |
| 00:30:59,630 --> 00:31:04,030 | |
| lymphocyte help وبالتالي مافيش Antibody production | |
| 353 | |
| 00:31:04,030 --> 00:31:08,070 | |
| أو B cell functioning يعني مافيش شيء this disease | |
| 354 | |
| 00:31:08,070 --> 00:31:10,310 | |
| may persist for a few months و | |
| 355 | |
| 00:31:12,600 --> 00:31:17,900 | |
| بالكتير لغاية سنتين it's not x-linked and should | |
| 356 | |
| 00:31:17,900 --> 00:31:24,240 | |
| be distinguished from x-linked disease خلال وجود | |
| 357 | |
| 00:31:24,240 --> 00:31:29,460 | |
| ال normal number of B cells immunization should | |
| 358 | |
| 00:31:29,460 --> 00:31:33,800 | |
| not be given during this period common variable | |
| 359 | |
| 00:31:33,800 --> 00:31:38,120 | |
| immunodeficiency من الإسم it's characterized by | |
| 360 | |
| 00:31:38,120 --> 00:31:39,280 | |
| غياب | |
| 361 | |
| 00:31:43,570 --> 00:31:51,510 | |
| ماشي او reduction في ال serum IgG و IgA و بيكون في | |
| 362 | |
| 00:31:51,510 --> 00:31:57,890 | |
| عنده IgM normal او low و طبعا في normal او low | |
| 363 | |
| 00:31:57,890 --> 00:32:03,950 | |
| برفر ال B cell number ال B cell fails to mature | |
| 364 | |
| 00:32:03,950 --> 00:32:09,650 | |
| into plasma cellوالمرضى have recurrent respiratory | |
| 365 | |
| 00:32:09,650 --> 00:32:13,550 | |
| and gastrointestinal infection treatment by | |
| 366 | |
| 00:32:13,550 --> 00:32:22,850 | |
| intravenous immunoglobulin selective | |
| 367 | |
| 00:32:22,850 --> 00:32:29,160 | |
| immunoglobulin deficiencyالـ IgA-Deficiency غالبا | |
| 368 | |
| 00:32:29,160 --> 00:32:31,860 | |
| مرضى بس يكون عندهم recurrent viral or bacterial | |
| 369 | |
| 00:32:31,860 --> 00:32:35,300 | |
| infection, celiac disease, defective absorption | |
| 370 | |
| 00:32:35,300 --> 00:32:39,060 | |
| and repel اللي هو عبارة عن الـ celiac or maybe | |
| 371 | |
| 00:32:39,060 --> 00:32:43,720 | |
| entirely asymptomatic، treatment بده pro-the | |
| 372 | |
| 00:32:43,720 --> 00:32:47,360 | |
| spectrum antibiotic، IgM-Deficiency، rare | |
| 373 | |
| 00:32:47,360 --> 00:32:52,300 | |
| disorders وطبعاالمرضى بيعانوا من recurrent and | |
| 374 | |
| 00:32:52,300 --> 00:32:54,740 | |
| severe infection with polysaccharide antigen | |
| 375 | |
| 00:32:54,740 --> 00:32:59,840 | |
| -encapsulated organism زي pneumonia أو pneumococci | |
| 376 | |
| 00:32:59,840 --> 00:33:05,040 | |
| and hemophilus inflammation ال disorders of T and | |
| 377 | |
| 00:33:05,040 --> 00:33:11,780 | |
| B interaction زي الhyper IgM syndrome وهو عبارة عن | |
| 378 | |
| 00:33:11,780 --> 00:33:18,150 | |
| hyper X-linked hyper IgM syndromePresent with | |
| 379 | |
| 00:33:18,150 --> 00:33:22,530 | |
| recurrent respiratory infection من أول سنة إلى | |
| 380 | |
| 00:33:22,530 --> 00:33:27,670 | |
| سنتين من العمر ميتيشن موجودة في CD40-ligand جين | |
| 381 | |
| 00:33:27,670 --> 00:33:32,010 | |
| الموجود على الإكسي كرومزوم اللي يبقى بيقدي إلى | |
| 382 | |
| 00:33:32,010 --> 00:33:37,890 | |
| CD40-ligand وبالتالي في الشيء | |
| 383 | |
| 00:33:40,910 --> 00:33:45,050 | |
| سيجنال ترانزدكشن يعني مافيش فانكشن CD40 لجن برت | |
| 384 | |
| 00:33:45,050 --> 00:33:50,390 | |
| برت مع CD40 موجود على سطح بي لينفوسايت وطبعا this | |
| 385 | |
| 00:33:50,390 --> 00:33:57,810 | |
| interaction rescue the B cell from apoptosis X | |
| 386 | |
| 00:33:57,810 --> 00:34:00,990 | |
| -linked lymph proliferative disease Duncan | |
| 387 | |
| 00:34:00,990 --> 00:34:08,070 | |
| syndrome وهذه بيصير في عدم قدرة عند ال T cell to | |
| 388 | |
| 00:34:08,070 --> 00:34:13,700 | |
| regulate B cell growthهو بيعتبر major part of the | |
| 389 | |
| 00:34:13,700 --> 00:34:19,820 | |
| underlying disease | |
| 390 | |
| 00:34:19,820 --> 00:34:24,220 | |
| يعني this mechanism deficiency هو الأساس في | |
| 391 | |
| 00:34:24,220 --> 00:34:29,730 | |
| الموضوع ال lymphoma incidence is highوانتقل للـ | |
| 392 | |
| 00:34:29,730 --> 00:34:35,090 | |
| phagocytic function فقط | |
| 393 | |
| 00:34:35,090 --> 00:34:40,930 | |
| للتذكير الفاجوسيتك فانكشن يعني إذا كان فيه خلل في | |
| 394 | |
| 00:34:40,930 --> 00:34:48,910 | |
| خطوات الفاجوسيتس فقط للتذكير الفاجوسيتس خطوات | |
| 395 | |
| 00:34:48,910 --> 00:34:54,620 | |
| الفاجوسيتس هي أدهيجينثم ال diabetes ثم ال | |
| 396 | |
| 00:34:54,620 --> 00:35:00,340 | |
| chemotaxis ثم الفاجو سايتوسيث ثم ال killing و | |
| 397 | |
| 00:35:00,340 --> 00:35:07,080 | |
| التالي في غياب في كل مرحلة من المراحل هذه يؤدي لها | |
| 398 | |
| 00:35:07,080 --> 00:35:11,280 | |
| كل مرحلة is combined or is associated with certain | |
| 399 | |
| 00:35:11,280 --> 00:35:18,460 | |
| disease ماشي اذا صار فيه برضه الخلل مضري معناته كل | |
| 400 | |
| 00:35:18,460 --> 00:35:22,550 | |
| المراحل اللي قد تليها واقفةوإذا كان الخلل حدث | |
| 401 | |
| 00:35:22,550 --> 00:35:27,990 | |
| مثلًا مثلًا في اللاد مثلًا مثلًا مثلًا مثلًا مثلًا | |
| 402 | |
| 00:35:27,990 --> 00:35:29,070 | |
| مثلًا مثلًا مثلًا مثلًا مثلًا مثلًا مثلًا مثلًا | |
| 403 | |
| 00:35:29,070 --> 00:35:32,470 | |
| مثلًا مثلن مثلن مثلن مثلن مثلن مثلن مثلن مثلن مثلن | |
| 404 | |
| 00:35:32,470 --> 00:35:33,030 | |
| مثلن مثلن مثلن مثلن مثلن مثلن مثلن مثلن مثلن مثلن | |
| 405 | |
| 00:35:33,030 --> 00:35:37,450 | |
| مثلن مثلن مثلن مثلن مثلن مثلن مثلن مثلن مثلن مثلن | |
| 406 | |
| 00:35:37,450 --> 00:35:43,450 | |
| مثلن مثلن مثلن مثلن | |
| 407 | |
| 00:35:44,920 --> 00:35:49,480 | |
| LAD هو برنامج group of disorders بيتميز من بيصير | |
| 408 | |
| 00:35:49,480 --> 00:35:52,560 | |
| فيه خلل في ال interaction ما بين ال leukocyte مع | |
| 409 | |
| 00:35:52,560 --> 00:36:00,660 | |
| ال vascular endothelial cells و طبعا هاي المراحل | |
| 410 | |
| 00:36:00,660 --> 00:36:04,700 | |
| طبعا جالوا فيه اكتر من مرحلة لل LAD disease و | |
| 411 | |
| 00:36:04,700 --> 00:36:09,240 | |
| قسموها لتلت مراحل LAD 1, LAD 2, LAD 3 و زي ما | |
| 412 | |
| 00:36:09,240 --> 00:36:13,400 | |
| انتوا شايفين في LAD 1 بيصير فيه خلل في ال diabetes | |
| 413 | |
| 00:36:15,830 --> 00:36:20,730 | |
| في الهد اتنين بيصير فيه خلل في ال adhesion في الهد | |
| 414 | |
| 00:36:20,730 --> 00:36:23,330 | |
| التلاتة بيصير فيه خلل في ال activation اللي هي | |
| 415 | |
| 00:36:23,330 --> 00:36:27,450 | |
| الفاجو سيتوسس بيصير فيها خلل ماشي اليوم بيصير فيه | |
| 416 | |
| 00:36:27,450 --> 00:36:33,290 | |
| خلل في واحد اللي هي ال adhesion أول واحدة بيصير | |
| 417 | |
| 00:36:33,290 --> 00:36:38,130 | |
| فيه خلل في كل هذه المراحل ماشي فاليوم بيصير فيه | |
| 418 | |
| 00:36:38,130 --> 00:36:41,270 | |
| خلل في أخر واحدة اللي هي ال activationطبعا بيحصل | |
| 419 | |
| 00:36:41,270 --> 00:36:51,110 | |
| فيه أمراض زي الـ Chediac Higashi Syndrom وCDD هاي | |
| 420 | |
| 00:36:51,110 --> 00:36:53,510 | |
| اللي هاد واحد حضر عن الـ autosomal recessive | |
| 421 | |
| 00:36:53,510 --> 00:36:58,870 | |
| disease مهابينج تو كروموزومانوم 22 المرضى عندهم ال | |
| 422 | |
| 00:36:58,870 --> 00:37:03,690 | |
| effect ان هتكون subunits of integrin molecule وهذا | |
| 423 | |
| 00:37:03,690 --> 00:37:09,210 | |
| بيقدي إلى اللي هو انه adhesion to feel adhesion | |
| 424 | |
| 00:37:09,210 --> 00:37:12,740 | |
| and migrationof all white blood cells in the army | |
| 425 | |
| 00:37:14,860 --> 00:37:18,980 | |
| في الأدواح المرضى يحصل عندهم current soft tissue | |
| 426 | |
| 00:37:18,980 --> 00:37:22,760 | |
| bacterial infection ويزيد الوقت blood cell count | |
| 427 | |
| 00:37:22,760 --> 00:37:27,540 | |
| لكن بدون bus formation يعني مافي activity ومافيش | |
| 428 | |
| 00:37:27,540 --> 00:37:31,060 | |
| كل الأخمات اللي ورا ال adhesion او ال effecting | |
| 429 | |
| 00:37:31,060 --> 00:37:35,140 | |
| wound healing lymphocyte function is also affected | |
| 430 | |
| 00:37:35,140 --> 00:37:39,500 | |
| due to the lack of lymphocyte functioning antigen | |
| 431 | |
| 00:37:39,500 --> 00:37:44,860 | |
| one expressionالـ Add to قلنا بيصير فيه defect in | |
| 432 | |
| 00:37:44,860 --> 00:37:50,180 | |
| selecting الـ legend والـ underlying defect اللي | |
| 433 | |
| 00:37:50,180 --> 00:37:54,400 | |
| هو Add to بيصير | |
| 434 | |
| 00:37:54,400 --> 00:37:58,700 | |
| فيه أيضًا خلال الفيلم فيوكوز متع Polism وده بيقد | |
| 435 | |
| 00:37:58,700 --> 00:38:03,140 | |
| إلى غياب فيوكوز ليت ال legend for selecting to be | |
| 436 | |
| 00:38:03,140 --> 00:38:08,560 | |
| bind وده مثل أو صورة حلوة لهذا الموضوع الاد واحد | |
| 437 | |
| 00:38:08,560 --> 00:38:18,290 | |
| في الشيءهي عبارة عن اتيجينماشي لد اتنين عبارة عن | |
| 438 | |
| 00:38:18,290 --> 00:38:24,270 | |
| rolling Selected تكون غير رقم تلاتة اللي هو ال | |
| 439 | |
| 00:38:24,270 --> 00:38:31,110 | |
| activation is defected طبعا هذا كله في لد واحد بس | |
| 440 | |
| 00:38:31,110 --> 00:38:36,070 | |
| فيه خلال في الارتباط و ال transmigration اللي هو | |
| 441 | |
| 00:38:45,690 --> 00:38:52,590 | |
| انتجرانيال او أوريجانيلي بيصير | |
| 442 | |
| 00:38:52,590 --> 00:38:57,750 | |
| في عندها قلة في ال interest of the organism و this | |
| 443 | |
| 00:38:57,750 --> 00:39:00,470 | |
| results in | |
| 444 | |
| 00:39:02,700 --> 00:39:11,060 | |
| فاجوزوم فرمشي أو فاجوزوم فرمشي او | |
| 445 | |
| 00:39:11,060 --> 00:39:11,740 | |
| فاجوزوم فرمشي او فاجوزوم فرمشي او فاجوزوم فرمشي او | |
| 446 | |
| 00:39:11,740 --> 00:39:17,280 | |
| فاجوزوم فرمشي او فاجوزوم فرمشي او فاجوزوم فرمشي او | |
| 447 | |
| 00:39:17,280 --> 00:39:22,200 | |
| فاجوزوم فرمشي او فاجوزوم | |
| 448 | |
| 00:39:23,380 --> 00:39:26,020 | |
| مخلوق كبير من المخلوق المخلوق المخلوق المخلوق | |
| 449 | |
| 00:39:26,020 --> 00:39:27,180 | |
| المخلوق المخلوق المخلوق المخلوق المخلوق المخلوق | |
| 450 | |
| 00:39:27,180 --> 00:39:30,020 | |
| المخلوق المخلوق المخلوق المخلوق المخلوق المخلوق | |
| 451 | |
| 00:39:30,020 --> 00:39:31,620 | |
| المخلوق المخلوق المخلوق المخلوق المخلوق المخلوق | |
| 452 | |
| 00:39:31,620 --> 00:39:33,240 | |
| المخلوق المخلوق المخلوق المخلوق المخلوق المخلوق | |
| 453 | |
| 00:39:33,240 --> 00:39:38,680 | |
| المخلوق المخلوق المخلوق | |
| 454 | |
| 00:39:38,680 --> 00:39:40,940 | |
| المخلوق المخلوق المخلوق المخلوق المخلوق المخلوق | |
| 455 | |
| 00:39:40,940 --> 00:39:46,160 | |
| المخلوق المخلوق المخلوق المخلوق المخلوق | |
| 456 | |
| 00:39:48,980 --> 00:39:53,040 | |
| وبالتالي بيحصل فيه عملية phagocytosis لكن مافيه | |
| 457 | |
| 00:39:53,040 --> 00:39:57,600 | |
| killing وبيحصل فيه intracellular survival | |
| 458 | |
| 00:39:57,600 --> 00:40:03,060 | |
| فالorganism بيؤدي لgranuloma formation ميوتيشن | |
| 459 | |
| 00:40:03,060 --> 00:40:06,380 | |
| وجدوها في ال enzymes which catalyzes the | |
| 460 | |
| 00:40:06,380 --> 00:40:10,800 | |
| respiratory burst وبيحصل | |
| 461 | |
| 00:40:10,800 --> 00:40:17,120 | |
| فيه قلة في الintracellular killing محصلة او غياب | |
| 462 | |
| 00:40:19,320 --> 00:40:22,700 | |
| وبنلاحظ هذه الحالات في أول شهر أو أول سنتين من | |
| 463 | |
| 00:40:22,700 --> 00:40:31,400 | |
| العمر بيصير عنده recurrent infection حتى أضعف ال | |
| 464 | |
| 00:40:31,400 --> 00:40:35,660 | |
| microbes مرة | |
| 465 | |
| 00:40:35,660 --> 00:40:40,720 | |
| أخر interferon جامع receptor deficiency بيصير فيه | |
| 466 | |
| 00:40:40,720 --> 00:40:43,900 | |
| mutation في ال interferon عائلة أخرى من عائلات ال | |
| 467 | |
| 00:40:43,900 --> 00:40:48,030 | |
| receptorبيؤدي الى inability of a monocyte to | |
| 468 | |
| 00:40:48,030 --> 00:40:53,450 | |
| respond to Gamma Interferon وبالتالي | |
| 469 | |
| 00:40:53,450 --> 00:40:56,430 | |
| في الsecretion of the tumor necrosis factor Alpha | |
| 470 | |
| 00:40:56,430 --> 00:41:01,850 | |
| ال patient are susceptible to weakly pathogenic | |
| 471 | |
| 00:41:01,850 --> 00:41:03,290 | |
| mycobacteria | |
| 472 | |
| 00:41:06,330 --> 00:41:10,090 | |
| هو الـ Natural Killer Cell Deficiency اللي نعرفه | |
| 473 | |
| 00:41:10,090 --> 00:41:15,130 | |
| بياب الـ Anky cell بيؤدي الى الـ Impaired | |
| 474 | |
| 00:41:15,130 --> 00:41:19,090 | |
| Allograft Rejection مفيش شيء الـ Impairment | |
| 475 | |
| 00:41:19,090 --> 00:41:24,930 | |
| Rejection And is linked to higher susceptibility | |
| 476 | |
| 00:41:24,930 --> 00:41:29,170 | |
| to viral disease و بيصير فيه metastasis from tumor | |
| 477 | |
| 00:41:31,140 --> 00:41:36,500 | |
| الـ Ankyl cells seen in Skid some T and phagocytic | |
| 478 | |
| 00:41:36,500 --> 00:41:40,540 | |
| cell disorder and in X-linked lymphoprotective | |
| 479 | |
| 00:41:40,540 --> 00:41:47,360 | |
| syndrome الأوراق | |
| 480 | |
| 00:41:47,360 --> 00:41:56,880 | |
| اللي بتتعلق بال complement يعني ال ..الـ | |
| 481 | |
| 00:41:56,880 --> 00:42:00,180 | |
| complement component الأولانية مهمة جدًا for | |
| 482 | |
| 00:42:00,180 --> 00:42:07,300 | |
| obsolete زي C3P غالبًا deficiency of complement is | |
| 483 | |
| 00:42:07,300 --> 00:42:14,700 | |
| associated with SLE disease وهي ال complement | |
| 484 | |
| 00:42:14,700 --> 00:42:22,740 | |
| يأسكي بميكانزم classical و alternative طبعًا | |
| 485 | |
| 00:42:22,740 --> 00:42:32,270 | |
| زي ما انتوا شايفيناللي هو سواء كان alternative او | |
| 486 | |
| 00:42:32,270 --> 00:42:38,110 | |
| lectin pathway او even classical غيابها بيدي لديها | |
| 487 | |
| 00:42:38,110 --> 00:42:42,330 | |
| diseases مختلفة ففي البداية infections with | |
| 488 | |
| 00:42:42,330 --> 00:42:46,630 | |
| encapsulated microorganismبقدم شوية robotoid | |
| 489 | |
| 00:42:46,630 --> 00:42:51,190 | |
| disease واسئل ايه تحت اذا كان في الماك او ال | |
| 490 | |
| 00:42:51,190 --> 00:42:56,830 | |
| memory attack complex بصير فيه نيسيريا جنورية او | |
| 491 | |
| 00:42:56,830 --> 00:43:03,260 | |
| نيسيريا مفكشةأي مريض بـ Deficiency of C1,4,2 اللي | |
| 492 | |
| 00:43:03,260 --> 00:43:07,160 | |
| هو البدايات من الـ Classical Pathway أو C3 | |
| 493 | |
| 00:43:07,160 --> 00:43:11,100 | |
| Deficiency نفسها بيصير فيه زيادة في الـ Infection | |
| 494 | |
| 00:43:11,100 --> 00:43:14,180 | |
| أو الـ Encapsulated Microorganism حسب الجدول اللي | |
| 495 | |
| 00:43:14,180 --> 00:43:18,080 | |
| شوفناه طوي زي Streptococcus pneumonia و الـ Biogen | |
| 496 | |
| 00:43:18,080 --> 00:43:24,720 | |
| و الانفلونزا و أزمة رومانسية بسبب تنظيف غير صحيح | |
| 497 | |
| 00:43:24,720 --> 00:43:25,480 | |
| للمعاملات الاميونية | |
| 498 | |
| 00:43:29,040 --> 00:43:33,640 | |
| الـ Deficiency of the man knows by the lectin | |
| 499 | |
| 00:43:33,640 --> 00:43:38,100 | |
| بيأدى الى طبعا الموجود على ال surface of microbe | |
| 500 | |
| 00:43:38,100 --> 00:43:45,040 | |
| with or without antibody and activate the | |
| 501 | |
| 00:43:45,040 --> 00:43:50,240 | |
| classical pathwayأيضًا بيؤدي غيابه إلى risk of | |
| 502 | |
| 00:43:50,240 --> 00:43:56,380 | |
| infection and SLE-like symptoms غياب C3 associated | |
| 503 | |
| 00:43:56,380 --> 00:44:04,700 | |
| with symptoms خصوصًا ال complications تبعتها غياب | |
| 504 | |
| 00:44:04,700 --> 00:44:08,480 | |
| ال lead complement component في الآخر يعني ال MAC | |
| 505 | |
| 00:44:08,480 --> 00:44:14,160 | |
| زي C56789 اللي بيكونوا ال MAC اللي هو membrane | |
| 506 | |
| 00:44:14,160 --> 00:44:21,270 | |
| attack complexIt's directly related to the primary | |
| 507 | |
| 00:44:21,270 --> 00:44:27,010 | |
| defect against gram-negative bacteria خصوصا | |
| 508 | |
| 00:44:27,010 --> 00:44:30,450 | |
| نيسيرياDefective | |
| 509 | |
| 00:44:30,450 --> 00:44:34,010 | |
| control of the complement component يعني حتى ال | |
| 510 | |
| 00:44:34,010 --> 00:44:37,930 | |
| control اذا صار فيه deficiency زي ال hereditary | |
| 511 | |
| 00:44:37,930 --> 00:44:43,850 | |
| angioedema مشهورة كتير غياب C1 esterase inhibitor | |
| 512 | |
| 00:44:45,150 --> 00:44:51,370 | |
| بيدى الى اللى هو ال hereditary angioedema طبعا | |
| 513 | |
| 00:44:51,370 --> 00:44:54,590 | |
| كمية كبيرة من ال vasoactive peptide patient | |
| 514 | |
| 00:44:54,590 --> 00:44:59,370 | |
| suffering from localized edema و it's life | |
| 515 | |
| 00:44:59,370 --> 00:45:03,850 | |
| threatening when it occurs in the larynx اه طبعا | |
| 516 | |
| 00:45:03,850 --> 00:45:07,230 | |
| بيصير فيه اش edema في ال larynx و التانى بيصير فيه | |
| 517 | |
| 00:45:07,230 --> 00:45:12,730 | |
| obstruction لل airway pathway treatment | |
| 518 | |
| 00:45:12,730 --> 00:45:19,410 | |
| غالباavoid trauma and infusion of C1 esterase | |
| 519 | |
| 00:45:19,410 --> 00:45:25,350 | |
| inhibitor.الجليكوزين الفسفة دي ال enacetol protein | |
| 520 | |
| 00:45:25,350 --> 00:45:33,270 | |
| deficiency نعرف ان هذا بلعب دور كبير في ال as | |
| 521 | |
| 00:45:33,270 --> 00:45:35,670 | |
| anchored protein | |
| 522 | |
| 00:45:37,690 --> 00:45:44,190 | |
| هذا عبارة عن عائلة من البروتينات والجليكوزيه | |
| 523 | |
| 00:45:44,190 --> 00:45:48,530 | |
| الفوسفات ديال الناسيتوه الانكولات is expressed على | |
| 524 | |
| 00:45:48,530 --> 00:45:51,110 | |
| ال membrane of RBCs lymphocyte granulocyte | |
| 525 | |
| 00:45:51,110 --> 00:45:54,590 | |
| endothelial cell and epithelial cell معظم الخلايا | |
| 526 | |
| 00:45:55,790 --> 00:45:58,450 | |
| طبعا DC protein هم عبارة عن البروتينات حماية | |
| 527 | |
| 00:45:58,450 --> 00:46:02,930 | |
| للخلايا من ال complement activity وبالتالي غيابهم | |
| 528 | |
| 00:46:02,930 --> 00:46:12,770 | |
| يؤدي إلى غياب ال destruction of cell طبعا | |
| 529 | |
| 00:46:12,770 --> 00:46:20,730 | |
| كل الخلايا تتأثر في هذا الموضوع وبالتالي the أيضا | |
| 530 | |
| 00:46:22,580 --> 00:46:26,300 | |
| بيصير فيه hemolysis for all these cells طبعا أشهر | |
| 531 | |
| 00:46:26,300 --> 00:46:29,800 | |
| هذه للغاية اللي هو ال PNH في ال PNH طبعا ال | |
| 532 | |
| 00:46:29,800 --> 00:46:33,760 | |
| patient بيصير فيه غياب لل enzyme required for | |
| 533 | |
| 00:46:33,760 --> 00:46:39,840 | |
| production of GBI اللي هو وبالتالي مافيش GBI عنكم | |
| 534 | |
| 00:46:39,840 --> 00:46:43,680 | |
| ال protein وبالتالي مافيش بروتينات حماية عشان هيك | |
| 535 | |
| 00:46:43,680 --> 00:46:50,200 | |
| بزيد ال susceptibility to hemolysisفي الـ Chronic | |
| 536 | |
| 00:46:50,200 --> 00:46:53,580 | |
| course of B.N.H. Intravascular Hemolysis or CARE | |
| 537 | |
| 00:46:53,580 --> 00:46:59,060 | |
| طبعا في الكدنى وهذا بيقدى الى اللى هوا activation | |
| 538 | |
| 00:46:59,060 --> 00:47:03,700 | |
| of complement يعنى فى degeneration للتشو أو damage | |
| 539 | |
| 00:47:03,700 --> 00:47:08,740 | |
| للتشو ونتوقف | |
| 540 | |
| 00:47:08,740 --> 00:47:12,520 | |
| هنا لنستكمل | |
| 541 | |
| 00:47:12,520 --> 00:47:14,220 | |
| فى مرحلة قادمة ان شاء الله | |