الملخص العربي : الهدف وخطة البحث : متابعة قياسات الطحال والوريد البابي والوريد الطحالي و قياسات اللويحات الدمويه وخلايا الدم البيض والحمر في مرضى تضخم الطحال النشط بعد العلاج الإشعاعي للطحال ( المجموعه 1) و مقارنه تلك القياسات بالمرضى الذين تلقوا علاج تدعيمى مساعد من غذاء وفيتامينات( المجموعه 2). المرضى وطرق البحث : تمت متابعة 19 مريضا بتضخم الطحال النشط ( 10 مرضى من المجموعه 1 لمده من 9 إلى 18 شهراً و9 مرضى من المجموعه 2 لمده من 4 إلى 6 شهور). تم العلاج الإشعاعي للطحال بمعدل مرتين إسبوعياً لمدة 3 أسابيع بجرعه كليه 9جراى. إستخدم جهاز الفحص بالموجات الصوتيه والدوبلر الملون في قياسات الطحال والوريد البابي والوريد الطحالي في بداية المتابعه وبعد 3 و 6 شهورلمرضى المجموعتين. وتم متابعة التغيرات فى قياسات اللويحات الدمويه والخلايا االبيض والحمر فى الدم بعد إسبوعين و4 و 8 أسابيع و 3 و 6 و9 شهور وبعد عام وعام ونصف. أظهرت النتائج زيادات ذات دلاله إحصائيه في حجم الطحال وقطر الوريدالطحالي في مرضى المجموعه 2 مقارنة بنقص في تلك القياسات ذي دلاله إحصائيه في المجموعه 1 ولم يتغير قطر الوريد البابي بدرجه ذات دلاله إحصائيه في المجموعتين. زاد عدد اللويحات الدمويه بدرجه ذات دلاله إحصائيه في كل فترات متابعة امجموعه 1بينما قلت في مرضى المجموعه الثانيه بدرجه غير ذات دلاله إحصائيه. ظهر نقص في متوسط حجم اللويحات (MPV) ومدى إتساع توزيعها(PDW) في مرضى المجموعه 1 في نهاية المتابعه بدرجه ذات دلاله إحصائيه بينما قلا في المجموعه 2 بدرجه غير ذات دلاله إحصائيه. لوححظت زيادات ذات دلاله إحصائيه في عدد الخلايا الحمر ومستوى الهيموجلوبين في المجموعه 1 بعد 6 و9 شهور بالترتيب بينما نقصت الخلايا الحمر بدرجه ذات دلاله إحصائيه في المجموعه بعد 6 شهور. لم تظهر تغيرات ذات دلاله إحصائيه في عدد الخلايا البيض. وأظهرت التغيرات في حجم الطحال علاقه إيجابيه ذات دلاله إحصائيه مع قطر الوريد الطحالي و علاقه عكسيه ذات دلاله إحصائيه مع التغيرات فى عدد الويحات الدمويه. وأظهرت التغيرات في قطر الوريد الطحالى علاقه إيجابيه ذات دلاله إحصائيه مع MPV. ولم تلاحظ عقب العلاج الإشعاعي سوى القليل من الأعراض الجانبيه العارضه. بإختصار يمكن إستنتاج ان العلاج الإشعاعي لتضخم الطحال النشط يعتبر مبشراً وقليل السميه يجب البحث في تخصيص جرعة إشعاع لكل مريض على حده حسب حالته الإكلينيكيه. . وكانت التغيرات في قطر الوريد الطحالي هى الأكثر علاقه بمعدلات موت وتجديد اللويحات الدمويه وخلايا الدم الحمرويمكن دراسة أهميتها في تضخم الطحال النشط مستقبلاً.

Abstract Objectives: (1) To investigate the dysregulation of apoptotic modulators in type I and type II diabetes. Serum levels of soluble Fas (sFas), sFas ligand (sFasL), tumor necrosis factor-alpha (TNF-) and its soluble receptor I (sTNF-RI), thiobarbituric acid reactive substances (TBARS) and nitric oxide (NO) were determined. (2) To evaluate the relationships of these bio-indices with glycemic control, disease complications (diabetic neuropathy) and duration. Patients and Methods: Sixty diabetic patients (30 type I and 30 type II diabetes) were sub-classified into non-complicated diabetic patients and patients with diabetic neuropathy. Patients were also subdivided according to glycemic control (HbA1c ≤ vs. > 11%) and diabetes duration (≤ vs. > one year). Each diabetic group was compared with its related age-matched control group. The serum levels of sFas, sFasL, TNF- and sTNF-RI were determined using specific ELISA assays. The serum levels of HbA1c, NO and TBARS were measured by colorimetric methods. Results: In types I and II diabetes, serum levels of sFas, TNF-, sTNF-RI, TBARS and NO were significantly higher in non-complicated and complicated diabetes than controls. These indices were found significantly higher in complicated than non-complicated diabetic patients Serum sFasL level was undetectable in controls while it was significantly higher in complicated than non-complicated type I patients. sTNF-RI level was significantly higher in patients with duration longer than 1 year than newly discovered cases. Significant increases in the investigated bio-indices were observed in types I and II diabetic patients with average and poor glycemic control compared to controls. Poor glycemic control showed significantly higher levels of TNF- in types I and II patients. TBARS and sFas levels were significantly higher in type II diabetic patients with poor glycemic control. There were significant positive correlations among the investigated biochemical indices and indicators of glycemic control, oxidative stress and duration of the disease particularly in type I diabetes. Conclusion: Type I and II diabetes are associated with dysregulated apoptotic modulators as shown by upregulation of sFas, sFasL, TNF-, sTNF-RI, TBARS and NO. Chronic complications of diabetes, bad glycemic control and increased duration of diabetes may be involved in the dysregulation of apoptotic modulators.

Abstract Objectives: (1) To investigate the dysregulation of apoptotic modulators in type I and type II diabetes. Serum levels of soluble Fas (sFas), sFas ligand (sFasL), tumor necrosis factor-alpha (TNF-) and its soluble receptor I (sTNF-RI), thiobarbituric acid reactive substances (TBARS) and nitric oxide (NO) were determined. (2) To evaluate the relationships of these bio-indices with glycemic control, disease complications (diabetic neuropathy) and duration. Patients and Methods: Sixty diabetic patients (30 type I and 30 type II diabetes) were sub-classified into non-complicated diabetic patients and patients with diabetic neuropathy. Patients were also subdivided according to glycemic control (HbA1c ≤ vs. > 11%) and diabetes duration (≤ vs. > one year). Each diabetic group was compared with its related age-matched control group. The serum levels of sFas, sFasL, TNF- and sTNF-RI were determined using specific ELISA assays. The serum levels of HbA1c, NO and TBARS were measured by colorimetric methods. Results: In types I and II diabetes, serum levels of sFas, TNF-, sTNF-RI, TBARS and NO were significantly higher in non-complicated and complicated diabetes than controls. These indices were found significantly higher in complicated than non-complicated diabetic patients Serum sFasL level was undetectable in controls while it was significantly higher in complicated than non-complicated type I patients. sTNF-RI level was significantly higher in patients with duration longer than 1 year than newly discovered cases. Significant increases in the investigated bio-indices were observed in types I and II diabetic patients with average and poor glycemic control compared to controls. Poor glycemic control showed significantly higher levels of TNF- in types I and II patients. TBARS and sFas levels were significantly higher in type II diabetic patients with poor glycemic control. There were significant positive correlations among the investigated biochemical indices and indicators of glycemic control, oxidative stress and duration of the disease particularly in type I diabetes. Conclusion: Type I and II diabetes are associated with dysregulated apoptotic modulators as shown by upregulation of sFas, sFasL, TNF-, sTNF-RI, TBARS and NO. Chronic complications of diabetes, bad glycemic control and increased duration of diabetes may be involved in the dysregulation of apoptotic modulators.

Abstract Objectives: (1) To investigate the dysregulation of apoptotic modulators in type I and type II diabetes. Serum levels of soluble Fas (sFas), sFas ligand (sFasL), tumor necrosis factor-alpha (TNF-) and its soluble receptor I (sTNF-RI), thiobarbituric acid reactive substances (TBARS) and nitric oxide (NO) were determined. (2) To evaluate the relationships of these bio-indices with glycemic control, disease complications (diabetic neuropathy) and duration. Patients and Methods: Sixty diabetic patients (30 type I and 30 type II diabetes) were sub-classified into non-complicated diabetic patients and patients with diabetic neuropathy. Patients were also subdivided according to glycemic control (HbA1c ≤ vs. > 11%) and diabetes duration (≤ vs. > one year). Each diabetic group was compared with its related age-matched control group. The serum levels of sFas, sFasL, TNF- and sTNF-RI were determined using specific ELISA assays. The serum levels of HbA1c, NO and TBARS were measured by colorimetric methods. Results: In types I and II diabetes, serum levels of sFas, TNF-, sTNF-RI, TBARS and NO were significantly higher in non-complicated and complicated diabetes than controls. These indices were found significantly higher in complicated than non-complicated diabetic patients Serum sFasL level was undetectable in controls while it was significantly higher in complicated than non-complicated type I patients. sTNF-RI level was significantly higher in patients with duration longer than 1 year than newly discovered cases. Significant increases in the investigated bio-indices were observed in types I and II diabetic patients with average and poor glycemic control compared to controls. Poor glycemic control showed significantly higher levels of TNF- in types I and II patients. TBARS and sFas levels were significantly higher in type II diabetic patients with poor glycemic control. There were significant positive correlations among the investigated biochemical indices and indicators of glycemic control, oxidative stress and duration of the disease particularly in type I diabetes. Conclusion: Type I and II diabetes are associated with dysregulated apoptotic modulators as shown by upregulation of sFas, sFasL, TNF-, sTNF-RI, TBARS and NO. Chronic complications of diabetes, bad glycemic control and increased duration of diabetes may be involved in the dysregulation of apoptotic modulators.

Abstract: BACKGROUND: Treatment of refractory severe chronic idiopathic thrombocytopenic purpura (ITP) is a difficult challenge especially in young patients who become steroid-dependant and when splenectomy is unfavourable or refused by some patients. Current strategy in ITP is to withdraw steroids and try any of the drugs that have been found effective in clinical trials. Dapsone, an inexpensive drug, was reported to be effective in the disease. RESULTS: Thirty consecutively patients (3 males&27 females) with resistant ITP with a mean age ± SE of 22.7 ± 4.2 years used 50 -100 mg of dapsone per day for 20 weeks with a mean of 12.2 ± 1.2 weeks. The mean platelet count (PC) increased significantly in all group from 26.8 ± 5.3 x 109/l. at start of the study to 60.9 ± 10.6 x 109/l after 4 weeks (P 0.01) and to 92.2 ± 15.9 x 109/l in the last visit (P 0.01). Patients with severe and moderate to severe thrombocytopenia had significant increase in PC after 2 and 4 weeks of dapsone therapy and in last visit PC ( P 0.001, P 0.01 and P 0.01 respectively for severe & P 0.05, P 0.05 and P 0.05 respectively for moderate to severe cases). Out of thirty patients 9(30 %) had a good response, 9 (30 %) a moderate response, and 12 (40%) a poor or no response. Good responders group had significantly lower age compared to bad responders group. Significant positive correlations were observed between platelet response to dapsone after 2 weeks and final platelet response in all patients (R = 0.461, P 0.05), in patients with poor response to treatment (R = 0.821, P 0.05) and patients with moderate to severe thrombocytopenia (R= 0.71, P 0.01). Significant inverse correlations were observed between duration of ITP and PC after 4 weeks treatment in all patients and in groups with moderate and moderate to severe thrombocytopenia. CONCLUSION: Dapsone is an effective and safe second line treatment in chronic ITP. Predictors of response are young age, PC more than 15 x 109 /L, short duration of disease and initial good response in the first 2 weeks.

Abstract: BACKGROUND: Treatment of refractory severe chronic idiopathic thrombocytopenic purpura (ITP) is a difficult challenge especially in young patients who become steroid-dependant and when splenectomy is unfavourable or refused by some patients. Current strategy in ITP is to withdraw steroids and try any of the drugs that have been found effective in clinical trials. Dapsone, an inexpensive drug, was reported to be effective in the disease. RESULTS: Thirty consecutively patients (3 males&27 females) with resistant ITP with a mean age ± SE of 22.7 ± 4.2 years used 50 -100 mg of dapsone per day for 20 weeks with a mean of 12.2 ± 1.2 weeks. The mean platelet count (PC) increased significantly in all group from 26.8 ± 5.3 x 109/l. at start of the study to 60.9 ± 10.6 x 109/l after 4 weeks (P 0.01) and to 92.2 ± 15.9 x 109/l in the last visit (P 0.01). Patients with severe and moderate to severe thrombocytopenia had significant increase in PC after 2 and 4 weeks of dapsone therapy and in last visit PC ( P 0.001, P 0.01 and P 0.01 respectively for severe & P 0.05, P 0.05 and P 0.05 respectively for moderate to severe cases). Out of thirty patients 9(30 %) had a good response, 9 (30 %) a moderate response, and 12 (40%) a poor or no response. Good responders group had significantly lower age compared to bad responders group. Significant positive correlations were observed between platelet response to dapsone after 2 weeks and final platelet response in all patients (R = 0.461, P 0.05), in patients with poor response to treatment (R = 0.821, P 0.05) and patients with moderate to severe thrombocytopenia (R= 0.71, P 0.01). Significant inverse correlations were observed between duration of ITP and PC after 4 weeks treatment in all patients and in groups with moderate and moderate to severe thrombocytopenia. CONCLUSION: Dapsone is an effective and safe second line treatment in chronic ITP. Predictors of response are young age, PC more than 15 x 109 /L, short duration of disease and initial good response in the first 2 weeks.

Hypertension is a well-recognized complication of autosomal recessive polycystic kidney disease (ARPKD). The renin-angiotensin system (RAS) is a key regulator of blood pressure; however, data on the RAS in ARPKD are limited and conflicting, showing both up- and down-regulation. In the current study, we characterized intrarenal and systemic RAS activation in relationship to hypertension and progressive cystic kidney disease in the ARPKD orthologous polycystic kidney (PCK) rat. Clinical and histological measures

Congenital hepatic fibrosis (CHF) is an important cause of morbidity and mortality in patients with Autosomal Recessive Polycystic Kidney Disease (ARPKD). The pathogenesis of CHF remains undefined. Several recent studies suggest that the reninangiotensin system (RAS) is an important mediator of progressive hepatic fibrosis, through activation of pro-fibrotic mediators, such as transforming growth factor-β (TGF-β). RAS activation has not previously been studied in CHF patients or animal models. The aim of the current study was to characterize RAS expression during the course of CHF in the PCK rat.