The aim of the study was to study the characteristics of systemic lupus erythematosus (SLE) in the Egyptian population, comparing it to other populations. We retrospectively studied 207 patients with SLE diagnosed between 1990 and 2005. We obtained clinical features and laboratory data and analyzed them statistically. We studied 151 female and 56 male SLE patients. The female to male ratio was 2.7 to 1 and the mean age at presentation was 10 +/- 2.7 years (range 2-16). The mean disease duration was 6.47 +/- 3.74 years. At diagnosis, musculoskeletal, constitutional and mucocutaneous manifestations were the commonest features. During follow-up, the prevalence of nephritis (67%), hematological manifestations (44.9%), photosensitivity (44%), arthritis (39%), malar rash (38.2%), serositis (32.9%) and neuropsychiatric manifestations (24.25%) increased significantly. Those whose age of onset of the disease was

Introduction. The presence of the deletion (D) allele in the angiotensin-converting enzyme (ACE) gene has been reported as a probable genetic risk factor for idiopathic nephrotic syndrome (INS), but its role in determining resistance to steroid therapy remains to be evaluated. Methods. Fifty-one patients were enrolled in the study and received oral steroids. The pattern of response to steroid therapy was determined. A group of 50 healthy adults served as a control group. The genotypes for ACE insertion (I)/D polymorphism were analysed using a PCR-based method. Results. Twenty patients were steroid sensitive (SS) and 31 were non-SS. The presence of hypertension at presentation was significantly related to steroid unresponsiveness. Among the SS group, the frequencies of the II, ID, and DD genotypes of the ACE gene were 20% (n=4), 65% (n=13), and 15% (n=3), respectively, while the frequencies among the non-SS group were 19.4% (n=6), 74.2% (n=23), and 6.5% (n=2), respectively. The differences between the two groups were not statistically significant (Chi square=0.59). Conclusion. The current study on Egyptian children with INS reveals no association between the ACE gene I/D polymorphism and clinical parameters, histological findings, and steroid resistance.

Nitric oxide production is reduced in renal disease, partially due to decreased endothelial nitric oxide production. Evidence indicates that nitric oxide deficiency contributes to cardiovascular events and progression of kidney damage. A polymorphism in intron 4 of the endothelial constitutive nitric oxide synthase (ecNOS) gene is a candidate gene in cardiovascular and renal diseases. We investigated a potential involvement of this polymorphism in chronic renal failure. A case-control study involved 78 children with chronic kidney disease (CKD) and 30 healthy controls. All participants were genotyped for the ecNOS4 polymorphism by the polymerase chain reaction (PCR). Dialyzed (maintenance hemodialysis) and conservative treatment children had significantly higher frequency of the aa genotype and ecNOS4a allele (P<0.05) compared with controls. The combined genotype aa+ab vs. bb comparison validated that a allele is a high-risk allele for end-stage renal disease (ESRD) (P<0.05). Serum nitric oxide level was found to be lower in carriers of the ecNOS 4a allele than in noncarriers (100.29±27.32 vs. 152.73±60.39 μmol/l, P=0.04). Interestingly, 85.95% of the ecNOS 4a allele ESRD patients were found hypertensive in comparison to the 60.67% patients of non noncarriers (bb genotype) (P=0.04). Also, 35.90% of the ecNOS 4a allele ESRD patients were found to have cardiovascular disease in comparison to the 5.13% patients of noncarriers (bb genotype) (P=0.01). On multiple linear regression analysis, a allele was independently associated with hypertension (P=0.03). There was a significantly higher frequency of the ecNOS4a allele carriers among CKD children, both on MHD and conservative treatment than in controls. This suggests that the ecNOS gene polymorphism may be associated with an increased risk of chronic renal failure.

As feto-placental vessels in humans are not innervated, regulation of vascular tone in the fetal extracorporeal circulation most likely depends on either circulating hormones or local paracrine mechanisms. However, the latter have not yet been fully investigated. The aim of our study was to characterize vasomotor behaviour of resistance stem villi arteries when challenged with various constrictor and dilator agents, with special emphasis on the physiological importance of endothelium. The latter is poorly characterized in this particular vascular bed in humans. Villous stem arterial rings (internal diameter 182 +/- 6 microns) were isolated under microscopy from term human placentae obtained after cesarean section. The vessels were mounted as ring preparations in an isometric myograph for tension measurements. Endothelium was removed from some of the rings by gentle insertion of a knotted human hair into the vascular lumen. Of the three vasoconstrictors tested, endothelin-1 (ET-1) showed the greatest potency, being 1,000 times more potent than serotonin and phenylephrine. The classical endothelium-dependent vasodilators, acetylcholine, adenosine diphosphate (ADP) and histamine, caused dose-dependent relaxation of the rings; an effect which was completely abolished by the removal of endothelium. Pre-treatment with the nitric oxide (NO) synthase inhibitor, N omega-nitro-L-arginine, also markedly reduced the endothelium dependent relaxant responses to ADP. By contrast, the vasodilatory response to sodium nitroprusside was not affected by endothelial removal. We conclude that i) ET-1 is a potent vasoconstrictor of the human placental vascular bed and ii) placental villous endothelial cells synthesize and release relaxing factor(s) which could possibly be NO.

Aim and MethodsWe investigated the association between polymorphisms of the angiotensin converting enzyme-1 (ACE-1) and angiotensin II type one receptor (AT1RA1166C) genes and the causation of renal disease in 76 advanced chronic kidney disease (CKD) pediatric patients undergoing maintenance hemodialysis (MHD) or conservative treatment (CT). Serum ACE activity and creatine kinase-MB fraction (CK-MB) were measured in all groups. Left ventricular mass index (LVMI) was calculated according to echocardiographic measurements. Seventy healthy controls were also genotyped.ResultsThe differences of D allele and DI genotype of ACE were found significant between MHD group and the controls (p = 0.0001). ACE-activity and LVMI were higher in MHD, while CK-MB was higher in CT patients than in all other groups. The combined genotype DD v/s ID+II comparison validated that DD genotype was a high risk genotype for hypertension .~89% of the DD CKD patients were found hypertensive in comparison to ~ 61% of patients of non DD genotype(p = 0.02). The MHD group showed an increased frequency of the C allele and CC genotype of the AT1RA1166C polymorphism (P = 0.0001). On multiple linear regression analysis, C-allele was independently associated with hypertension (P = 0.04).ConclusionACE DD and AT1R A/C genotypes implicated possible roles in the hypertensive state and in renal damage among children with ESRD. This result might be useful in planning therapeutic strategies for individual patients.

Uncuffed central venous catheters are both needed and useful for short-term hemodialysis. Vascular access for extended durations may be provided by cuffed tunneled catheters. Infection is the major serious concern with both uncuffed and cuffed catheters.

The aim of this study was to determine which endothelial factors were involved in the decrease of pulmonary vascular resistance at birth, and how they changed with maturation.Response of intrapulmonary artery rings precontracted with prostaglandin F 2a were studied from piglets aged <2 h, 2±3 day, 10 day and adult pigs for pharmacological responses to acetylcholine (ACh) and cromakalim (CMK) in the presence and the absence of the nitric oxide synthase (NOS) inhibitor, N v -nitro-L-arginine (L-NA), the adenosine triphosphate sensitive potassium (KATP) channel blocker, glibenclamide and the endothelin (ET)-A receptor antagonist, BQ123. In situ hybridization and immunochemistry studies were performed in lung tissues of the same animals in order to determine the expression of NOS and ET.There was a small contractile effect of ACh in the newborn. Relaxation to ACh, which was blocked by L-NA and reduced by glibenclamide, only appeared from the age of 3 days. The significantly greater relaxation to CMK in rings without endothelium (p<0.05) was abolished by BQ123 in the newborn, and then disappeared by 2 days of age. Glibenclamide had a greater inhibitory effect on relaxation induced by CMK at 10 days than in the newborn and 2 days old piglets. NOS expression was low in pulmonary arteries of the newborn and increased by 2 days of age whereas the converse was seen with ET expression.It is concluded that: 1) relaxant response to acetylcholine was absent at birth and appeared at 2 days; 2) the reduced relaxant response to cromakalin in rings with endothelium at birth could be blocked by BQ123; and 3) the expression of endothelin decreased whereas the expression of nitric oxide synthase increased from birth to 2 days of age. Eur Respir J 2000; 15: 158±165. Pulmonary arterial pressure and resistance are high at birth, and decrease rapidly thereafter due to structural [1±3] and functional [4,5] changes. The underlying mechanisms of these modifications are not fully understood, but nitric oxide (NO) is known to play a role. NO [6±8] is released from the lungs of foetal and newborn animals [5,9,10]. It reduces basal tone in utero and contributes to the postnatal fall in pulmonary vascular resistance. However, endothelium-dependent relaxation to various agonists is either absent [11] or very weak [12] until the third day after birth. Then it rapidly increases during the first week of life [11,12]. These observations suggest that immediately after birth the interactions between the endothelium and the underlying smooth muscle may be different from those in adult porcine pulmonary vasculature.Vasodilators such as NO [13], prostacyclin [14], bradykinin [15], endothelium-derived hyperpolarizing factor (EDHF) [16], and vasoconstrictors such as endothelin (ET)-1 [17] and thromboxane [18] are important factors modulating pulmonary vascular tone. ET-1 plasma levels are higher at birth than at 3 days and in adults [19]. Although endothelium-dependent relaxation to acetylcholine (ACh) is absent, the amount of cyclic guanosine...

A greater percentage of steroid-resistant patients were found in our patients compared with those in other studies. Response to immunosuppressives was different from other studies, probably due to differences in the priority of selection for immunosuppressive therapy.