فهرست مطالب

Nephro-Urology Monthly - Volume:2 Issue:3, 2010
  • Volume:2 Issue:3, 2010
  • تاریخ انتشار: 1389/04/01
  • تعداد عناوین: 15
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  • Selective Inhibition of α-Galactosidase A with Antisense Oligodeoxynucleotide in Mesangial Cells: A Renal Cellular Model for Fabry Disease
    Utsumi K., Itoh K., Hirama A., Ueda K., Sakamaki M., Kaneko T., Yamazaki M., Komaba Y., Katsura Ki, Iino Y., Katayama Y Page 3
    Background And Aims
    Fabry disease is an X-linked lysosomal storage disease resulting from deficient activity of the enzyme α-galacotsidase (α-Gal) A. Accumulation of glycosphingolipids, especially globotriaosylceramide, leads to various organ damage in Fabry disease. Recently, replacement with recombinant α-Gal A has become available for the treatment of this disease. However, the pathogenic mechanism of this disease, which is the accumulation of glycosphingolipids, is still unknown. Understanding the pathogenesis of Fabry disease may allow more efficient treatments. We examined whether the selective inhibition of α-Gal A with phosphorothioate antisense oligonucleotides could be used as a renal cellular model for Fabry disease..
    Methods
    Phosphorothioate antisense oligonucleotides designed to hyrbridize to sites on the human α-Gal A mRNA were tested for inhibition of α-Gal A expression in human mesangial cells. α-Gal A activity was measured using an artificial substrate, 4-methylumbelliferyl-α-D-galactoside..
    Results
    Two antisense oligonucleotides selectively inhibited α-Gal A activity to below 20% of the mean control activity. These oligonucleotides did not affect other lysosomal enzyme activities..
    Conclusions
    These data indicate that phosphorothioate oligonucleotides are capable of selectively inhibiting α-Gal A expression. It may be a useful model for renal mesangial cells in Fabry disease..
    Keywords: Fabry Disease, α galactosidase A, Antisense Oligodeoxynucleotide, Globotriaosylceramide
  • Rostami Z., Lessan, Pezeshki M Page 387
  • Mookadam F., Kheir Me, Alharthi M., Moustafa Se, Kazimier Fm, Mcbain R., Mookadam M., Tajik Ja, Milliner Ds Page 390
    Background And Aims
    Primary hyperoxaluria (PH) is a rare genetic disorder characterized by calcium oxalate nephrocalcinosis leading to renal failure. When renal function is impaired, plasma oxalate concen-tration increases and extra-renal oxalate deposition occurs. Vascular involvement includes vasculitis and ischemic related morbidity and death. The prevalence of vascular involvement is not known with only a few cases described in the literature. A systematic overview of the literature was performed to identify the demographics, clinical presentation and outcomes of such patients..
    Methods
    A computerized search of MEDLINE (Jan 1950 to May 30, 2007), EMBASE (Jan 1988 to May 2007) and CINAHL (Jan 1982 to May 30, 2007 identified twenty three cases of vascular involvement..
    Results
    The majority of those reported in the literature are young females (74%). Vascular deposition of oxalate crystals presents as skin vasculitis; limb or mesenteric ischemia or gangrene. Stroke is uncommon..The lower limbs are affected more than upper limbs followed by mesenteric vessels. Vasculitic skin manifes-tations occur in 52% of cases. Death occurred in 8 (38%) of cases (63% abdominal gangrene/sepsis; 37% cardiac)..
    Conclusions
    Little information exists on vascular involvement in PH. Morbidity and mortality is high (death and limb loss occurring in 2/3 of patients). Awareness of the vascular presentation is thus important to allow a better understanding. Prospective reporting via a national or international registry on optimal management of vascular involvement in PH is needed..
    Keywords: Nephrocalcinosis, Urolithiasis, Aminotransferase, Vasculitis, Vasculopathy, Skin Rash, Amputa, tion, Gangrene, Hepatorenal Transplant
  • Okasha K., Shahba A., Noor, Eldeen Nm, Hassan Am, Saadany He, Bendary Ae Page 401
    Background And Aims
    Systemic lupus erythematosus (SLE) is an autoimmune disease in which the complement system plays a crucial role in its pathogenesis. Mannan-binding lectin (MBL) is a recognition molecule of the lectin pathway of complement activation. The presence of several polymorphisms at the promoter and coding regions of the MBL-2 gene determines alterations at MBL serum concentration. MBL variant alleles that lead to low serum levels and/or functional deficits of MBL are postulated to contribute to the susceptibility of SLE. Moreover, the influence of MBL variation on antibody production and renal involvement in SLE patients remains controversial.
    Objectives
    MBL serum level and genotypes were studied in our SLE Egyptian patients with evaluation of its role in auto antibodies production and lupus nephritis development..
    Methods
    MBL genotypes and serum level were screened in a case control study included 30 SLE patients as well as 30 healthy controls. MBL polymorphism at exon 1 codons 54 and 57 was detected by PCR using sequence-specific priming (SSP) and serum MBL level was determined by ELISA technique..
    Results
    There was predominance of AA genotype (80%) in control group. Genotype frequencies of MBL variants in patients with SLE showed significant differences when compared with controls (AA 53.3% vs 80%, P=0.03, OR = 0.29 and AO+OO 46.6% vs 20%, P = 0.03, OR = 3.5, respectively). Serum MBL in our SLE patients (900 ng/ml) was significantly lower than that of the control group (2750 ng/ml, P = 0.00) with positive correlation with low MBL genotypes. SLE patients with mutant alleles were more likely to produce anti dsDNA (92.8% vs 75%, OR = 4.3) and anti-Smith (35.7% vs 18.7%, OR = 2.3). Patients carrying MBL-low genotypes have an increased risk of development of lupus nephritis than those carrying MBL-high genotype (64.7% vs 35.2%, P = 0.02, OR= 2.4)..
    Conclusion
    MBL gene polymorphism associated with low MBL serum levels that were found with significantly increased frequency in our SLE patients may be one of the genetic factors that determine the susceptibility to develop lupus nephritis..
    Keywords: Mannose, Binding Lectin (MBL), Systemic Lupus Erythematosus (SLE), Lupus Nephritis (LN), Gene Polymorphism
  • Reddy Pe, Manohar Sm, Reddy Sv, Bitla Ar, Vishnubhotla S., Lakshmi Narasimha Sp Page 414
    Background And Aims
    Patients on maintenance hemodialysis are at an increased risk of cardiovasculardisease. Oxidative stress has a negative impact on endothelial functions producing endothelial dysfunction which contributes to cardiovascular risk. FRAP assay has been shown to be a simple cost effective tool for estimating antioxidant capacity. Hence the present study was taken up to evaluate total antioxidant capacity as ferric reducing ability of plasma (FRAP) and malondialdehyde (MDA), in patients during hourly intervals of a single hemodialysis session. During hemodialysis, FRAP is subjected to alterations, due to intradialytic changes in various molecules that contribute FRAP. This study was aimed to assess the utility of FRAP as a measure of the antioxidant capacity during hemodialysis..
    Methods
    Twenty seven patients with end stage renal disease on maintenance hemodialysis were recruitedinto the study. Time course changes in plasma MDA, FRAP, uric acid, total bilirubin, vitamin C and vitamin E were evaluated. Statistical evaluation of changes in the biochemical parameters during the whole period of dialysis was done using Friedman''s test. Linear regression using generalized estimating equations (GEE) model for repeated measures was applied to study the association between parameters on intradialytic FRAP..
    Results
    Intradialytic increase in plasma MDA (p<0.01), decrease in FRAP (<0.01) and uric acid (p<0.001) levels were found. Uric acid was found to have significant association with FRAP as found by GEE (p<0.001)..
    Conclusions
    A single session of hemodialysis contributes substantially to oxidative stress. Decreasedintradialytic FRAP levels can be due to a decrease in uric acid levels. Assessment of antioxidant status in hemodialysis patients by FRAP method may not truly represent the actual antioxidant status, as changes in uric acid levels are reflected in FRAP levels. Hence measurement of individual antioxidants would give a better picture of the antioxidant status during hemodialysis..
    Keywords: Oxidative stress, Hemodialysis, Malondialdehyde, Total Antioxidant Capacity
  • Fatima N., Zaman Mu, Kamal S., Hameed A Page 422
    Background And Aims
    Cisplatin is an effective agent for the treatment of solid tumors and nephrotoxicity isits serious side effect. This nephrotoxicity is dose dependent and results in debilitating renal failure. The aim of this study was to determine the early nephrotoxicity by serial estimation of glomerular filtration rate (GFR) using plasma two sample clearance method (PSC2) as the reference method and compared with other methods..
    Methods
    Thirty three patients (26 males and 7 females; mean age 45 ±14 yrs) with different solid tumors whowere scheduled to receive cisplatin (mean dose of 114 ±23 mg/m2) in each cycle. GFR was determined simultaneously by 6 methods; (1) 24 Hours Creatinine Clearance (24HCrCl); (2) Gamma camera uptake method after 99mTc-diethylene triamine pentaacetic acid (DTPA) injection (Gates method); (3) Predicted creatinine clearance by Modification of Diet and Renal Diseases (MDRD); (4) Cockcroft-Gault''s equation (CG''s); (5) PSC 2; (6) Plasma one Sample Clearance method after 99mTc-DTPA injection (PSC 1). PSC 2 was chosen as reference method..
    Results
    Three out of thirty three patients developed severe acute nephrotoxicity (GFR <50 ml/min). In remaining 30 patients, average fall in GFR after 6th cycle of cisplatin was 43.86 ± 16.10 ml/min/1.73 m2 (p <0.001) estimated by PSC2 which was more significant after 3rd cycle. Correlation coefficient (r) for PSC1, Gates, 24HCrCl, CG''s and MDRD methods were 0.95, 0.91, 0.51, 0.59 and 0.67 respectively..
    Conclusions
    GFR is the most sensitive indicator of early cisplatin induced nephrotoxicity and PSC1 andGates method are reliable substitute of PSC2 method. CG''s, MDRD and 24 HCrCl methods are not reliable for detection of early nephrotoxicity. It is recommended that serial GFR should be estimated before and after every cisplatin dose, if not possible, than at least after the third cycle for precise detection of early nephrotoxicity..
    Keywords: Cisplatin, Nephrotoxicity, GFR, PSC2, Gates, PSC1, MDRD, CGs
  • Kamel Eg, El Minshawy O Page 431
    Background And Aims
    End Stage Renal Disease (ESRD) has various causes that differ according topatient''s country. In Egypt it is a huge health problem with high prevalence in El-Minia governorate as 308per million population suffer from it. The aim of the work is to investigate the possible causes of ESRD with an unknown etiology in El-Minia Governorate..
    Methods
    A total number of 216 patients with an unknown etiology of ESRD as well as 220 controls were interviewed using a structured questionnaire including information about environmental factors predicted to cause ESRD such as occupation, pesticide handling, and source of drinking water..
    Results
    Distribution of patients by residence showed that most patients lived in rural areas (76%), comparedto 57% in controls (p<0.001). Drinking unsafe water was reported by 72% of patients and 48% of controls (p<0.001). Family history of renal disease was found among 10% of patients and 4% of controls (p<0.001). Exposure to pesticides and using herbs for the treatment were more among patients than controls (52% Vs 14% respectively) and (34% Vs 6% respectively) (p<0.001)..
    Conclusions
    ESRD with an unknown etiology may be attributed to environmental factors suhc as drinking unsafe water, exposure to pesticides and using herbs for treatment. Educational programs for common people should be strengthened. Lead pipes water supplies should be changed. Use of any herbs should be prohibited except under the supervision of Ministry of Health..
    Keywords: ESRD, Unknown Etiology, Environmental Factors
  • Ijoma C., Arodiwe E., Ulasi I., Anisiuba B Page 438
    Background And Aims
    A high prevalence of pericardial effusion and low prevalence of pericardial thickeninghave been documented in end stage renal disease (ESRD) especially in patients undergoing dialysis. The aim of the study was to investigate the presence of pericardial disease in predialysis chronic kidney disease (CKD) patients and to determine relationship of pericardial disease with the aetiology of CKD..
    Methods
    This is a prospective cross-sectional conducted in Enugu, Nigeria. Eighty eight consecutive predialysis CKD patients, and forty four age and sex-matched control subjects were studied using twodimensional echocardiography..
    Results
    Fifty six percent of the patients had pericardial disease while 44% did not. Pericardial disease was detected as early as stage 3 CKD. Of the 88 patients studied 15.9% had pericardial effusion only, 29.5% had pericardial thickening only and 10.2% had a combination of both. Majority (81.8%) were in ESRD. Systolic blood pressure, diastolic blood pressure, CKD stage, and serum phosphate correlated positively with pericardial disease. Haemoglobin concentration, glomerular filtration rate, and serum albumin correlated negatively with the presence of pericardial disease. The aetiology of CKD did not correlate with the presence of pericardial disease. Regression analysis showed that only serum haemoglobin predicted the presence of pericardial effusion..
    Conclusions
    Pericardial disease is common in Nigerian patients with CKD at first evaluation and occurs as early as stage 3 CKD. Pericardial thickening is more prevalent than pericardial effusion. Pericardial effusion is predicted by low haemoglobin concentration. Echocardiography to detect pericardial disease should be part of routine investigation of patients with CKD..
    Keywords: Pericardial Effusion, Pericardial Thickening, Chronic Kidney Disease, Echocardiography
  • Landsmann S., Schwarz S., Haeberle L., Engehausen Dg, Wullich B., Krause Fs Page 447
    Background And Aims
    In advanced transitional cell cancer of the bladder a systemic chemotherapy isconsidered therapy of choice with palliative intent in spite of an unsatisfactory rate of remission. We tested a new chemotherapeutic agent, irinotecan, presenting with fair response rates in patients with advanced intestinal carcinomas, in animals with poorly differentiated transitional cell carcinomas (TCC)..
    Methods
    The effect of irinotecan was tested using single and multiple applications as well as in combination with docetaxel. Local tumor growth and body weight of mice were registered frequently. Histopathology of local tumor and previously defined organs were obtained after death. All data were proofed statistically..
    Results
    The animal groups are statistically significant different for the tumor size (p < 0.01). Post-hoc tests showed that the control group and the irinotecan monotherapy (p = 0.04), as well as the irinotecan/docetaxel group (p < 0.01) are significant different for a reduced tumor mass. No significance could be detected for regression rate and survival time of the animals..
    Conclusions
    The chemotherapy of irinotecan as monotherapy or in combination with docetaxel seems to be a promising alternative in the palliative treatment of advanced, metastatic transitional cell cancer of the bladder. Due to the significant influence of irinotecan on the reduction of tumor mass, we are planning a prospective randomised study to confirm these data in humans..
    Keywords: Bladder Cancer, Advanced Disease, Chemotherapy, New Approach, Irinotecan
  • Hibi H., Ohori T., Yamada Y., Honda N., Ito C., Sano M., Hashiba Y., Asada Y Page 455
    Background And Aims
    Microdissection testicular sperm extraction TESE (MD-TESE), which can improvesurgical sperm retrieval rate is widely applied in sperm retrieval surgery in non-obstructive azoospermic (NOA) patients. We assessed whether MD-TESE can improve sperm retrieval rate as well as Intracytoplasmic sperm injection (ICSI) results..
    Methods
    A retrospective comparative study involving 86 NOA patients who underwent 6-8 conventional multiple TESE (C-TESE) procedures was conducted. Another 116 NOA patients underwent MD-TESE. CTESE and MD-TESE were employed for the bilateral testis via a median raphe incision under spinal or local anesthesia with spermatic block..
    Results
    Motile sperm recovery was achieved in 26 (30.2%) and 30 (25.8%) patients with C-TESE and MDTESE,respectively. Collected sperm was cryopreserved. All couples in which motile sperm was recovered received ICSI utilizing frozen-thawed sperm samples. Overall pregnancy rates of the C-TESE and MD-TESE groups were 23% (6/26) and 40% (12/30), respectively. Abortion was not observed in either group and healthy deliveries resulted. Statistical differences in sperm recovery and pregnancy rate were not evident between the groups (P=0.495, P=0.182, respectively). Moreover, no successful pre-operative predictors of sperm recovery were identified..
    Conclusions
    These data demonstrated that MD-TESE does not improve sperm recovery rate; however, MD-TESE contributes to favorable pregnancy rate in NOA patients..
    Keywords: Non, Obstructive Azoospermia, Conventional TESE, Microdissection TESE, Sperm Recovery Rate, Pregnancy Rate
  • Doluoglu Og, Tatar Ca, Kutucularoglu G., Akdemir Ao, Canbay A., Diker E., Adsan O Page 462
    Background And Aims
    It has been shown that erectile dysfunction (ED) and coronary arterial disease (CAD) share predisposing pathophysiology and risk factors. We planned present study to determine ED as an early precursor of CAD using objective angiographic methods..
    Methods
    Twenty-two patients without any cardiac symptoms who referred for ED were assigned for thefirst group. Another 20 patients who were underwent the coronary angiographic (CAG) assessment and had CAD, and without any previous ED history were assigned to the second group. All patients were asked for their sexual intercourses during the last 12 months period. The degree of ED is determined by means of IIEF-EF questionnaire and penile doppler ultrasonography..
    Results
    CAD was diagnosed in 12 (54.5%) patients out of 22 whom had ED diagnosis without any cardiaccomplaints. There was no statistically significant difference between the serum parameters and risk parameters of the patients. In the second group, ED was diagnosed in 9 (45%) patients out of 20 who had been diagnosed with CAD after the coronary angiography..
    Conclusions
    CAD was present in high proportions among ED patients without any symptomatic cardiac disease. Our study depended on the objective criteria such as angiography and colour doppler ultrasound in the same patient group and thus we believe that it is unique study to support the idea that ED can be the indicator of CAD.
    Keywords: Coronary Artery Bypass Surgery, Erectile Dysfunction, Doppler Ultrasonography, PenileErection
  • Charafi S., Mbarki M., Bauza Ac, Prieto Rm, Oussama A., Grases F Page 469
    Background And Aims
    The scanning electron microscopy (SEM) and Fourier transform infrared spectrophotometry(FTIR) methods are particularly useful for the analysis of renal calculi due to their simplicity and the information they provide, and the results can guide therapeutic approaches and prevention of recurrence. The aim of this study was to compare SEM and FTIR as methods for renal calculus analysis..
    Methods
    Analysis of renal calculi from the Tadla-Azilal region (Morocco) were performed by optical stereomicroscopy, followed by FTIR or SEM. The results obtained by the two methods were compared..
    Results
    Both methods clearly showed a predominance of calcium oxalate monohydrate (COM; 54%) in renal calculi from the study region, while calcium oxalate dihydrate calculi represented only 13.5%. Purine calculi were almost as frequent as phosphate calculi (24.3%), and struvite calculi comprised 8.1%. The high prevalence of COM calculi could be the result of several factors including sedentary lifestyles and eating habits, the latter involving a major contribution to oxalates from frequent consumption of tea..
    Conclusions
    The results showed good agreement between the two methods, which provided appropriate information on the composition and crystal structure of renal calculi..
    Keywords: Renal Stone Analysis, Scanning Electron Microscopy, Fourier Transform Infrared Spectrophotometry, Etiology, Composition Prevalence
  • Singh O., Gupta Ss, Girish G., Aggarwal G., Mathur Rk Page 476
    Background And Aims
    Urolithiasis has an overall prevalence of 2-3% worldwide. Extracorporeal shockwave lithotripsy (ESWL) is one of the noninvasive procedures used for the management of the same. The outcome after ESWL depends on various factors which include position, size, number and composition of stones, double-J (DJ) stenting, presence of hydronephrosis etc. The present study is aimed to assess the effect of stone related factors such as size, number, location; renal factors such as hydronephrosis and patient factors such as obesity, habitus, on the outcome of ESWL in urolithiasis..
    Methods
    A set of 280 patients with calculi in the kidney and ureter were subjected to ESWL during theperiod from April 2005 to September 2008, with pre and post-ESWL serial studies of the calculi, including x-ray and ultrasonography (USG). The results were subsequently assessed for the influence of prognostic factors on the outcome, using the Chi-Square test..
    Results
    Overall stone free rate was 63%. Upper pole renal stones had a significant clearance over the lower pole stones (p=0.01). Clearance of ureteric stones of <1cm in size, was significantly greater than that of those >1cm (p=0.03). Patients with hydronephrosis showed a statistically significant clearance over patients without hydronephrosis (p=0.034). Clearance of single stone was also significantly better than that of multiple stones (p=0.001). Sixteen patients underwent DJ stenting pre-ESWL, of which 8 were cleared of the stone, without significant difference..
    Conclusions
    The size, position, and number of calculi had a significant impact on the outcome after ESWL. Calculi with hydronephrosis obtained better clearance..
    Keywords: Extracorporeal Shock Wave Lithotripsy, Urinary tract, Stones
  • Rana T., Langford Ae, Akoh Ja Page 481
    Background And Aims
    The severity of organ shortage places transplant surgeons in the difficult position ofdeciding whether to use or discard marginal donor kidneys. This case details the successful use of a pelvic kidney with a short ureter and multiple vessels from a controlled non heart beating donor and reviews relevant literature..Case Report: A 69 year old lady received the right kidney from a 48 year old female controlled non heart beating donor. The donor kidney had three arteries (one of which was damaged) on one aortic patch, and an extremely short (7cm), thin-walled and narrow lumen (2-3 mm) ureter, all of which combined to make the transplantation technically challenging and the postoperative course complicated. In the post transplantation period, there were difficulties in establishing adequate transplant drainage due to frequent nephrostomy tube and ureteric stent dislodgement, and sepsis. The patient was discharged home on day 62 with a glomerular filtration rate of 31 ml/min and a creatinine of 148 μmol/L, and these were 51 ml/min and 97 μmol/L nine months postoperatively..
    Conclusions
    This case demonstrates that it is possible to extend the boundaries for accepting marginal kidneys for transplantation, after discussing the risks of the procedure with the patient. Ureteric stenting, intense postoperative monitoring of the transplant and early intervention in the event of ureteric complications must be applied to ensure success.Implantation of marginal donor kidneys with damaged or short ureters should be considered if a good renal function is likely..
    Keywords: Pelvic Kidney, Marginal Donor, Renal Transplantation, Ureteric Stricture, Ureteric Stent
  • Hajari Ar, Kulkarni Jn Page 486
    Incidental detection of two primaries call for oncosurgeon''s own judgment to decide the best therapeutic approach as no guidelines exist given the rarity of condition..This is the sixth reported case of incidental renal cell cancer in a patient of endometrial cancer. Renal cell cancer was detected on preoperative Magnetic Resonance Imaging in a patient who presented as postmenopausal bleeding and histology proven endometrial carcinoma. Both primaries were simultaneously treated surgically. Final histology confirmed dual primaries with uterine primary being endometrioid adenocarcinoma stage Ic, estrogen progesterone receptor positive; where as renal primary was clear cell carcinoma stage Ib, estrogen and progesterone receptor negative..Estrogen receptors have been identified in Hamster and mouse kidneys as well as in renal cell carcinoma tissues. High plasma estrogen found in some patients of synchronous renal and endometrial cancer may partly explain the association of these two primaries, though not in all cases. Increased serum leptin levels and a common low penetrance susceptibility gene have been reported in both these cancers..
    Keywords: RCC, EndometrialCancer, Estrogen Receptors