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Objective: To assess the frequency of complications in image-guided percutaneous nephrostomy and to identify common sources of error., Methods: The study was carried out at the Sindh Institute of Urology and Transplantation, Karachi, between November 2006 and May 2007. Patients of all age groups between 1 and 80 years were included using nonprobability convenience sampling technique. Those suffering from obstructive uropathy due to various causes were diagnosed by imaging modalities like ultrasound, computed tomography scan, conventional X-ray and contrast studies. It also included cases where percutaneous nephrostomy was used to temporarily divert urine in the presence of urinary tract leaks and fistula so that healing may occur. Patients with uncorrectable bleeding diathesis were excluded. Nephrostomies performed for supplementary procedures were also excluded. One-month follow-up was performed by means of direct communications and using various imaging modalities. SPSS 12 was used for statistical analysis., Result: Three hundred patients enrolled in the study. The procedure was successful in all encounters. The complications were categorised as early and late complications. Early complications were sepsis in 6 (2%) patients, retroperitoneal haematoma in 5 (1.6%) patients, bleeding in 2 (0.6%), and urinoma in 1 (0.3%). Late complications included catheter blockage in 15 (5%) patients, and dislodgement of catheter in 7 (2.3%). Total early complications were noted in 14 (4.66%) patients, and there were 22 (7.33%) late complications., Conclusion: Percutaneous nephrostomy is a safe, simple and cost-effective technique with low morbidity and no major life-threatening complications.

Objective: To evaluate the experience of 37 years regarding etiology, complications and evolving practice of the management of stricture urethra patients at the Sindh Institute of Urology and Transplantation, Karachi., Methods: The retrospective descriptive study included 1600 cases of stricture urethra admitted to the Urology Section of the institute from 1972 to 2009. Files of all patients were reviewed; age, gender, site of stricture, etiology, diagnostic methods, clinical symptoms and management, as well trends and patterns were noted., Results: There were 1600 patients of whom 1595 (99.4%) were males and 5 (0.4%) were females with age ranging from 14-80 years. Pelvic fracture urethral disfraction defects (n = 655; 49.5%) and fall astride (n = 123; 9.2%) were the commonest causes. Of the total, 92 percent presented with retention of urine. In the first decade, rail-road and dilatation was the mainstay of treatment. In the second decade, rail-road, dilatation and direct visual internal urethrectomy were the mainstay, but in the last 5 years, urethroplasty replaced the old methods., Conclusion: Trauma is the main cause of stricture. Over the years, urethroplasty and direct visual internal urethrotomy are the mainstay of management. Stricture clinic plays an important role in the early diagnosis of complications of stricture urethra and in the rehabilitation of these patients.

Nephrotic syndrome is a common pediatric glomerular disease associated with heavy proteinuria. Since, the angiotensin converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism is a putative genetic risk factor for NS, in this study, ACE (I/D) polymorphism was analyzed in 268 NS and 223 control samples by a PCR-based method. The genotypic and allelic frequencies were determined and the association between ACE I/D polymorphism and NS was evaluated. The frequency distribution of the II, ID and DD genotypes was 82 (30.6%), 128 (47.8%) and 58 (21.6%) in the NS patients and 9 (4.0%), 171 (76.7%) and 43 (19.3%) in the control samples respectively. In the Pakistani pediatric NS population, the II genotypic and allelic frequencies were found to be significantly associated with the disease (OR=6.755; C.I=3-14.9). No significant association was found between this polymorphism and the response to standard steroid therapy. Thus, in contrast to reports from other parts of the world, the II genotype was found to be significantly associated with NS in the Indian and Malay populations and in the Pakistani population described here. To our knowledge, this is the first report from Pakistan describing the association of the ACE I/D polymorphism with pediatric NS. On the basis of these results, it is suggested that analysis of the ACE (I/D) polymorphism should be performed for the early diagnosis in the high risk NS patients in South Asia., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Objective: To review 17 years experience of the stone clinic with incorporating the changes in practice over the years and to report the benefits of stone clinic in a developing country., Methods: The SIUT Stone clinic was established in 1990 with installation of HM4 Lithotriptor. This clinic is run jointly by a Urologist, Dietitian, Nephrologist, Biochemist and Radiologist. From 1990 - 2007, about 38,749 stone patients received treatment with ESWL (55%), PCNL (6.0%), URS (15.5%), litholopaxy 4.0% and open surgery 19.7%. These patients after treatment were followed in the stone clinic with stone analysis and 24 hours urine metabolic studies where indicated. Dietary and oral hydration programme combined with medical therapy was also instituted. Recurrence rate was noted in those patients who were advised diet modification, oral hydration and medical treatment. Complications of stone disease were documented during the follow-up period., Results: In ESWL group 8226 patients were followed in the stone clinic for 5 years. In this group, 185 (2.2%) had recurrence of renal calculi. In PCNL group 1306 patients were followed, and 16 (1.1%) had recurrence. In the open surgery group of 1294 patients, 17 (1.3%) had recurrence of renal calculi. The complications of stone disease noted were renal failure and infections in 162 (1.5%) patients, during the follow-up period., Conclusion: This study shows the beneficial effect of a stone clinic in a developing country and positive effect on the reduction of recurrence and complications of stone disease. Our experience promotes the need to open more specialized stone clinics in areas where stone disease is highly prevalent.

Objective: To evaluate the utility of serum CD30 (sCD30) levels as predictor of early acute graft rejection in live related renal transplant programme., Methods: This prospective study included 50 consecutive renal transplant recipients who received their first live related renal allograft at the Sindh Institute of Urology and Transplantation (SIUT) between October 2006 and March 2007. Blood samples were obtained one day before transplantation and on the third and fourteenth posttransplant days. Blood samples were also obtained from 50, age and sex matched healthy control individuals. Levels of serum sCD30 were measured by Enzyme Linked Immunosorbent Assay (ELISA)., Result: Donor-recipient blood group matching was identical in all patients. Pre-transplant lymphocyte crossmatch for T and B cells was negative, and panel reactive antibodies (PRA) were 0% for all recipients. The mean age of recipients was 31.6 +/- 10.23 years (range 5 to 55 years), while mean donor age was 32.74 +/- 8.48 years (range 21-50 years). Eleven (22%) recipients and donors were HLA identical while remaining (78%) were one haplotype match. Average serum sCD30 pre-transplant levels (37.8 +/- 4.97U/ml) were significantly higher than those of healthy individual's mean value of 8.48 +/- 4.97 U/ml, (P = 0.001). Eight (16%) patients developed acute rejection episode during this follow up period. Rejections were described and classified according to BANFF 97 classification., Conclusion: In this small single center study the serum levels of sCD30 did not show any significant difference between rejection and non rejection group in our transplant population.

Objective: To compare the outcome of Extra corporeal shockwave lithotripsy for a renal pelvic stone with and without JJ stent., Methods: A comparative cross sectional study was carried out at Sindh Institute of Urology and Transplantation from January 2007 to January 2008. Eighty patients with renal pelvic stone measuring 2cm +/- 2mm were selected for treatment with Extra Corporeal Shockwave Lithotripsy (ESWL). All of these patients were adults with normal renal function and had unilateral renal stones with negative urine cultures. Patients with renal failure and children were excluded. They were divided into two groups of 40 each. Group A patients underwent ESWL without a JJ stent and in Group B a JJ stent was placed before ESWL. SLX F2 electromagnetic ESWL machine was used to impart shock waves. 3000 shockwaves were given in a session. Both the groups were compared for renal colic, steinstrasse, fever, lower urinary tract symptoms (LUTS) emergency room visits and hospital admissions, stone clearance, number of ESWL sessions, auxilliary procedures, (percutaneous nephrostomy or ureterorenoscopy) and cost., Results: Ureteric colic occurred in 13 (32.5%) patients in group A and in 3 (7.5%) patient in group B. Steinstrasse developed in 4 (10%) patients with out JJ stent and in 3 (7.5%) patients with JJ stent. Fever was encountered in 1 (2.5%) patient in group A and in 3 (7.5%) patient in group B. Mean emergency room visits were 2.1 per patient in group A and 0.7 per patient in group B. Stone clearance occurred in 33 (82.5%) patients in group A and 31 (77.5%) in group B. In group B lower urinary tract symptoms were found in 50% versus 20% in group A. Auxillary procedure was performed in one (2.5%) patient each in both groups., Conclusion: Pre ESWL JJ stenting for a 2 cm +/- 2 mm renal stone was not beneficial in terms of steinstrasse, fever, stone clearance and number of ESWL sessions. However ureteric colic was significantly less in the stented group. Lower urinary tract symptoms (LUTS) was also significantly high in the patients having a JJ stent. The cost of the treatment doubled in the stented group which is an important factor in our country. JJ stenting does not prove to be a cost effective procedure when compared to the reduction in complications.

Objective: To determine the frequency of celiac disease (CD) in children with failure to thrive (FTT) and/ or persistent diarrhea (PD) not responding to conventional therapy., Design: Cross-sectional study., Place and Duration of Study: Gastroenterology Unit of Sindh Institute of Urology and Transplantation (SIUT), from January 2002 to January 2004., Patients and Methods: Forty nine children and adolescents with PD (defined as diarrhea greater than 14 days duration) and / or FTT (based on anthropometrical indicators, i.e. weight for length below the 5th centile) were included in the study. Demographic data, weaning practices, breast feeding and family history of CD were documented. Laboratory workup included tissue transglutaminase antibody IgA (tTGA), IgA and IgG antigliadin antibodies (IgA AGA and IgG AGA), HLA typing, upper gastrointestinal endoscopy (EGD) with distal duodenal biopsy (where allowed by parents) and anthropometric data. CD diagnosis was in accordance with the "Guidelines for the diagnosis of and treatment of CD for children" by NASPGHAN. Mann-Whitney U-test, Chi-square test and Fisher's exact test were used for analysis as applicable., Results: Forty nine patients (25 [51%] males) with FTT and or PD were included. The mean (+/-SD) age, height, weight and BMI were: 10.1+/-6.2 years (range, 1-20 years), 107.0 +/- 31.7 cm (45-180 cm), 19.04 +/- 12.5 kg (3-68 kg) and 16.12+/-12.58 kg/cm2 respectively. FTT was present in 30 (61%) patients. Thirty four (69%) children had a history of PD, 38 (77%) had a significant history of weight loss and 32 (65%) children had short stature. Majority of the children (88%) were breast fed. Weaning was started at 6 months of age in 40% and included mixed diet according to age. Protuberant abdomen was present in 26 (53%). Elevated tTGA was significantly (p < 0.001) more frequent in patients with CD. HLA-DQ2 and DQ8 haplotypes were positive in 18 (60%) of these patients. Thirty (61%) patients were positive for CD based on Marsh criteria. CD diagnosis was supported by positive tTGA and/ or AGA tests. Majority i.e. 28/30 patients had Marsh stage 3 disease, while 22/30 patients with CD also had PD. Four CD patients were found to suffer from protein calorie malnutrition (PCM)., Conclusion: Children who fail to thrive with or without PD may have CD. A positive tTGA test in these children is a useful aid in making a CD diagnosis. Majority of CD positive children were found to carry HLA DQ2 or DQ8 genes. Thus, HLA typing could be used to determine genetic disposition to CD. Majority of FTT had Marsh stage 3 changes on histopathology.

Objective: To compare the efficacy of Holmium: YAG laser and pneumatic lithoclast in treating ureteric calculi., Methods: The study included total of 100 patients divided into two equal groups of laser lithotripsy (LL) and pneumatic lithoclast (PL). Study was conducted between September 2006 and February 2007. Inclusion criteria were patients with a ureteric stone of size 1-2 cm and negative urine culture. An x-ray KUB was mandatory. IVU and CT pyelogram were also done when required. Procedures were done under general anaesthesia after a single dose of pre-operative antibiotic. A 7.5 Fr semi rigid ureteroscope was used for ureteroscopy in all cases. Holmium: YAG laser with 365 microm wide probe was employed in laser group and frequency was set between 5 and 10 Hz at a power of 10 to 15 W. Swiss lithoclast with single or multiple fire technique was used accordingly in PL group. Postoperatively patients underwent radiography and helical CT as required at 4th week of follow up to asses stone clearance., Results: The mean patient age in LL and PL group was 38 +/- 10 and 40 +/- 10 years respectively. The male to female ratio and stone size were similar between the groups. Stone migration up in pelvicalyceal system occurred in two patients of LL group while in eight patients of PL group. JJ Stent was placed in 5(10%) patients in laser group where as 13 (26%) patients required it in pneumatic lithoclast group. Stone free rate at 4 weeks was 92% in laser group as compared to 82% in pneumatic lithoclast group. Hospital stay was more than 24 hours in 2 patients of laser group as compared to 5 patients of pneumatic lithoclast group. Complication rate was 4% in LL group whereas it was 14% in PL group., Conclusion: Holmium: YAG laser lithotripsy is a superior technology compared to pneumatic lithoclast in terms of rate of stone clearance and complications, especially in upper ureteric stones.

Objective: To compare holmium Yag laser with lithoclast in patients with renal stones undergoing percutaneous nephrolithotomy (PCNL)., Methods: A comparative cross sectional study was performed on 60 patients undergoing PCNL. Patients with a 2.5 cm stone in renal pelvis, having preoperative negative urine culture, no coagulopathy and fit for general anaesthesia were included, pregnant females were excluded. They were randomly divided into 2 groups of 30 cases each depending on the energy source used. Group A included cases of PCNL subjected to laser treatment and group B had patients undergoing PCNL with pneumatic lithoclast. For each group duration of procedure, any per operative or postoperative complication, residual stone, duration of hospital stay and cost of each procedure were recorded., Results: The average operative time was 125.7 +/- 31.1 minutes in group A and 98.5 +/- 18.7 minutes in group B (P = 0.0001). The overall complication rate was similar in both groups being 13.3% in group A and 23.3% in group B. Residual stone was observed in 17% cases in group A and 13% cases in group B (P = 0.5). The mean postoperative hospital stay was 3.17 +/- 1.6 days in group A and 4 +/- 2.3 days in group B (P = 0.4). Cost analysis showed that the initial capital cost of equipment was 40,000 Euro in laser and 24,000 Euro in pneumatic lithoclast. However because of reuse of fiber the per procedure cost was 60 Euro in laser and 50 Euro in lithoclast group., Conclusion: In our experience we found that Holmium: YAG laser and pneumatic lithoclast are both effective and safe lithotriptors for percutaneous stone removal. More operating time was required in laser, more complications encountered with pneumatic lithoclast and a high initial cost of laser. However with increasing experience with laser, more promising results are expected with this new technology. The cost can be compensated by using it in other procedures especially at a public sector hospital.

Objective: To find the relationship between human leukocyte antigens (HLA) and cryoglobulin positivity in hepatitis C virus (HCV) infected individuals., Methods: Eligible individuals selected from pre and post renal transplant settings were divided into three groups. Group A (n = 301) consisted of normal controls, while group B (n = 200) comprised of pathological controls that were HCV antibody (anti-HCV) positive but negative for cryoglobulins. Group C comprised of 56 anti-HCV positive, cryoglobulin positive patients. HLA-A, -B and -DRB1 loci were typed by polymerase chain reaction (PCR) method and relationship between HLA antigens, anti-HCV status and cryoglobulinaemia was analyzed., Results: HLA-A*02, -B*57 and -DRB1*03 were more frequently found among group C members as compared to groups A and B. Only HLA-B* 57 occurrence reached statistical significance (14.3% versus 6% and 4%, corrected P-value = 0.045 and 0.012 and OR = 2.6 and 4 respectively) No differences in the distribution of HLA antigens were seen among healthy and pathological controls., Conclusion: The presence of HLA-B*57 confers susceptibility to cryoglobulinaemia in HCV infected patients in our population. HCV positive renal transplant recipients with these alleles should be monitored for cryoglobulin formation).

Objective: To document the prevalence of Helicobacter pylori (H. pylori), Hepatitis A virus (HAV), Hepatitis C virus (HCV), Hepatitis E virus (HEV) antibodies and Hepatitis B virus surface antigen (HBsAg), in the pediatric age group of low socioeconomic urban communities of Karachi and to identify risk factors associated with these infections., Design: Cross-sectional survey., Place and Duration of Study: Three selected squatter settlements of Karachi during April 2002 to December 2004., Patients and Methods: Three hundred and eighty children, ages 5 months to 15 years were investigated. Venous blood samples were collected and questionnaire filled on sociodemographic characteristics (family income, number of dependents in the family, area of living, number of people per room per house, and number of children sharing bed with parents and siblings). Gastrointestinal symptoms were recorded. Anti-HAV IgG (Hepatitis A virus IgG antibody), anti-HCV (Hepatitis C virus antibody), anti-HEV (Hepatitis E antibodies) and HBsAg, were analyzed by enzyme immunoassays (EIAs). Samples were also screened for anti-HIV1/2 (human immunodeficiency virus 1 and 2 antibodies by EIA. IgG antibodies against H. pylori were detected by immunochromatography., Results: A correlation between increasing age and seroconversion was seen for hepatotropic viruses. At 14 years and above,100% of the children were found to be positive for anti-HAV, 26% for anti-HEV, and 1.4%, for anti-HCV while HBsAg was positive in 1.9%. H. pylori infection did not show a significant increase with age. Both anti-HAV and anti-H. pylori were present simultaneously in 30% of the population investigated., Conclusion: With age, increasing number of children acquired antibodies against hepatotropic viruses and H. pylori. Occurrence of HBsAg and anti-HEV at a later age suggests horizontal, rather than vertical transmission.

Crescentic glomerulonephritis complicating the course of bacterial endocarditis carries a poor prognosis. Ideal treatment strategy is not clearly defined. In addition to antibiotic treatment, plasmapheresis and steroids have been used with variable results. Here we report a case of 40-year old female who was referred because of generalized body swelling and decrease urine output associated with low grade fever on and off for two to three months. She was diagnosed to have acute renal failure secondary to tricuspid valve endocarditis. Staph aureus was isolated from blood culture and renal biopsy showed crescentic glomerulonephritis. She received dialysis support and antibiotics and had complete recovery of renal function 6 weeks after initiation of therapy. Eradication of infection with antibiotics treatment may be sufficient for resolution of crescentic glomerulonephritis associated with infective endocarditis in some cases.

Biliary Atresia (BA) is a well-known entity and can present with multiple congenital anomalies. BA is one of the most common conditions in which pediatric liver transplant is performed. Identification of Biliary atresia with situs inversus (SI) has not been documented in Pakistan. We report two such cases. First was an eighty-day-old baby boy, icteric from day of birth. On further evaluation had dextrocardia, SI, gross hydronephrosis (HN) of left kidney and stasis at pelvi ureteric junction (PUJ). Liver biopsy showed biliary cirrhosis secondary to extra hepatic biliary atresia (EHBA). The second baby presented at two months of age. Ultrasound abdomen and hepatobiliary scintigraphy confirmed liver in left hypochondrium (SI) and findings suggestive of BA. Echocardiography confirmed SI with mesocardia. In this paper we have described the association of BA with SI in two patients presenting at the pediatric Gastroenterology, hepatology and nutrition unit.

Objective: To determine the pattern of excretion of total bilirubin IXa and IXb in the first meconium of newborn infants., Methods: First two newborns of varying gestational age were selected every week through random sampling from the neonatal unit. Of the 41 newborn infants selected 8 expired before meconium passage, hence the results are from 33 newborns. Meconium was collected and stored at -20 degrees C, protected by aluminium foil. Samples were defrosted, vortex mixed with equal amount of dimethyl-sulfoxide, centrifuged, and analyzed by HPLC., Results: Unconjugated Bilirubin-IXalpha and -IXbeta were identified and quantitative estimation of Bilirubin-IXa done. Bilirubin-IXb was greater than 50% of the total, in the first meconium of the newborn. Amount of bilirubin excreted in meconium was 29.2 - 90.8 mg [0.051 - 0.155 mmol] per sample of meconium passed. Amount was 9.7 mg/ Kg of body weight in term newborn and 12 mg/kg in preterm., Conclusion: The amount of bilirubin -IXb decreases and bilirubin-IXa increases with increasing gestational age. Newborns with birth asphyxia (BA) had significantly greater quantity of bilirubin in meconium, compared to infants without BA.

Objective: Evaluation of fractional excretion of Sodium, Potassium and Magnesium as indicators of cyclosporine (CsA) toxicity in de-novo renal transplant recipients., Methods: A prospective study was conducted on 59 live related renal allograft recipients. Fractional excretion(FE) of sodium (Na+), potassium (K+) and magnesium (Mg2+) were calculated on day 1, 3, 5 and 10 post transplant. Graft dysfunctions were evaluated by colour-Doppler, CsA levels and renal biopsy. Normal ranges were determined on 30 healthy subjects., Results: The mean creatinine on day 1 was 3.1 +/- 1.3 mg/dl and declined to 1.6 +/- 1.2 on day 10. FE of Na+, K+ and Mg2+ were 12 +/- 9%, 34 +/- 20% and 13 +/- 10% respectively on day 1 and reduced to 2.2 +/- 2%, 11 +/- 14% and 11 +/- 14% on day 10. Of the 59 recipients, 38 (64%) had uneventful recovery (group A), 21(36%) had graft dysfunction [6 acute rejection (group B) and 15 either acute tubular necrosis or high CsA(group C)]. In group A, on day 1, FENa+, FEK+ and FEMg2+ were 5 +/- 4%, 24 +/- 12% and 6.6 +/- 3% respectively and these declined to 1.2 +/- 0.6%, 4.6 +/- 0.7% and 6 +/- 3% respectively on day 10. Compared to group A, group C had significantly high values on day 1, FENa+ 15 +/- 8%, FEK+ 36 +/- 24% and FEMg2+ 21 +/- 10% (p < 0.0001) and on day 10, FENa+ 3.7 +/- 2.7%, FEK+ 20 +/- 15% and FEMg2+ 15 +/- 8% (p < 0.05). In the group B, day 1 and day 10 levels were FENa+ 6 +/- 3%, FEK+ 26 +/- 13% and FEMg2+ 7 +/- 2.8% and FENa+ 1.2 +/- 0.7%, FEK+ 4.2 +/- 0.5%, FEMg2+ 7 +/- 4% respectively. CsA levels and AUC did not correlate with CsA toxicity., Conclusion: FE of magnesium is a useful marker of CsA toxicity independent of CsA blood levels. FE studies can supplement renal biopsy findings.

Objective: To evaluate the effect of cyclosporine (CSA) on serum magnesium and its fractional excretion in renal transplant recipients., Methods: A cross sectional comparative study on 50 live related renal transplant recipients on CSA therapy with serum creatinine < 2.0 mg/dl and 30 healthy controls. Serum creatinine, magnesium and its fractional excretion and CSA levels were monitored. Patients were followed at 6 months., Results: The mean serum creatinine in patients was 1.41 +/- 0.42 mg/dl, cyclosporine 210 +/- 66 ng/ml at a dose of 4.8 +/- 1.4 mg/kg/day. The serum magnesium was 1.77 +/- 0.32 mg/dl vs 1.98 +/- 0.17 mg/dl in healthy controls (p < 0.05). Fractional excretion was 5.05 +/- 2.53% in patients vs 2.8 +/- 1.05% in controls (p < 0.05). No correlation was found between CSA levels (100-400 ng/ml) and serum magnesium (r = 0.053) or FEMg% (r = 0.215). Of the 50 recipients 27 (54%) had FEMg% in the control range. At 6 months follow up no difference in CSA levels was found between recipients with FEMg% in the normal range vs those with FEMg > 5%. However, serum creatinine increased from 1.42 +/- 0.30 mg/dl to 1.68 +/- 0.82 mg/dl (p < 0.05)., Conclusion: CSA therapy lowers serum magnesium as compared to healthy controls and there is marked increase in FEMg% in 50% of the patients. Patients with FEMg > 5% developed renal function deterioration. FEMg percent can thus be a good follow up marker of CSA chronic toxicity in stable transplant recipients.