Update on Surgical Management of Pediatric Urolithiasis.

Urolithiasis has always been a fascinating disease, even more so in children. There are many intriguing facets to this pathology. This article is a nonsystematic review to provide an update on the surgical management of pediatric urolithiasis. It highlights the pros and cons of various minimally invasive surgical options such as extracorporeal shockwave lithotripsy (ESWL), retrograde intrarenal surgery (RIRS), percutaneous nephrolithotomy (PCNL), laparoscopy, and robotics. This article also describes the various intracorporeal disintegration technologies available to fragment the stone, including the newer advancements in laser technology. It also emphasizes the cost considerations especially with reference to the emerging economies. Thus, this manuscript guides how to select the least-invasive option for an individual patient, considering age and gender; stone size, location, and composition; and facilities and expertise available.

Composition of urinary calculi in infants: a report from an endemic country.

Pediatric urolithiasis remains endemic in low-resource countries where infants constitute 17-40% of all children with urolithiasis. This study reports socio-economic factors, medical history and chemical composition of urinary stones in 976 infants of up to 2 years of age. Between 1992 and 2016, 976 infants presented to our institute with 1038 stones. Chemical composition of stones was analyzed by Fourier transformation infrared spectroscopy (FTIR). The mean age of infants was 19.5 ± 5.74 months with a M:F ratio of 5.5:1. Half (50%) of the infants were rural dwellers, 90% belonged to low socio-economic class and 70% were malnourished. A history of chronic diarrhoea was reported in 13% and urinary tract infections in 5%. The anatomic location was bladder in 487 (46.9%), kidney in 246 (23.6%), ureter in 142 (13.6%), urethra in 2 (0.2%) and 161 (15.5%) were passed spontaneously. Overall frequency of compounds in stones showed ammonium acid urate (AAU) in 772 (74.37%), calcium oxalate (CaOx) in 410 (39.5%). Uric acid (UA) in 119 (11.46%), calcium phosphate apatite (CaP) in 96 (9.25%), magnesium ammonium phosphate (Struvite) in 45 (4.34%), cystine in 12 (1.16%) and xanthine in 40 (3.85%). Frequency of compounds was similar in genders. Infants of age 0-6 months had higher frequency of UA (28%), CaOx (50%) and low AAU (61%) as compared to 11, 39 and 75%, respectively, in 7-24 months (p < 0.049) (p < 0.002) (p < 0.001). Nucleus of stones showed pure AAU in 63 and 43% on the surface due to addition of CaOx, struvite and CaP. Our study has shown that AAU is a major component of stones in infants where the main risk factors are poverty, malnutrition, diarrheal diseases and dehydration.

The association of urinary interferon-gamma inducible protein-10 (IP10/CXCL10) levels with kidney allograft rejection.

BACKGROUND: Interferon-gamma inducible protein-10 (IP-10/CXCL10) is a chemokine involved in the alloimmune response against kidney allograft. We aimed to investigate the association of urinary CXCL10 protein levels with rejection in renal transplant patients. METHODS: A total of 273 urine samples from (biopsy-proven) rejection and non-rejection patients and controls were included in this study. CXCL10 levels were analyzed for association with rejection. RESULTS: The data showed statistically significant differences in the CXCL10 levels between rejection vs. non-rejection (p < 0.001). Among the rejection groups, statistically significant differences for CXCL10 levels were found between ACR vs. NAD (p < 0.001), ACR vs. BLR (p = 0.019) and AVR vs. NAD (p = 0.009). Receiver Operating Characteristic (ROC) curve analysis of CXCL10 showed an area under the curve (AUC) of 0.74 with 72% sensitivity and 71% specificity at 27.5 pg/ml between rejection and non-rejection group. Kaplan-Meier curve analysis among different levels of CXCL10 showed a better rejection-free graft survival in patients with <100 pg/ml when compared to >200 pg/ml (38 ± 6 vs. 12 ± 1.0 weeks; log-rank p < 0.001) and 100-200 pg/ml (38 ± 6 vs. 22 ± 9 weeks; log-rank p = 0.442) concentration. CONCLUSION: The results indicate significantly increased levels of CXCL10 protein in the urine at the time of allograft rejection. This association of urinary CXCL10 protein levels with rejection could provide an additional tool for the non-invasive monitoring of allograft rejection.

Monocyte Chemoattractant Protein-1 (MCP-1/CCL2) Levels and Its Association with Renal Allograft Rejection.

BACKGROUND: CCL2 is a chemoattractant for monocytes/macrophages, T cells, and natural killer cells. It is shown to be involved in the immunological responses against renal allograft. This study was conducted to access the role of urinary CCL2 expression in predicting the rejection episodes in renal transplant patients. METHOD: A total of 409 urine samples included in this study. The samples were consisted of (a) biopsy-proven graft rejection (n = 165); (b) non-rejection (n = 93); (c) non-biopsy stable-graft (n = 42), and (d) healthy renal donors (n = 109). The samples were quantified for the CCL2 using the MCP-1/CCL2 ELISA kit. The data were analyzed using the Statistical Package for Social Sciences (SPSS®) and MedCalc® statistical software. RESULTS: Results showed that the CCL2 levels were significantly increased in rejection group when compared with the non-rejection, stable-graft, and control, P < 0.05. The receiver operating curve's characteristics illustrated that the urinary CCL2 level is a good predictor for graft rejection, with an area under the curve of 0.81 ± 0.03 with optimum sensitivity and specificity of 87% and 62%, respectively, at a cut-off value of 198 pg/mL. Kaplan-Meier curve also showed better cumulative rejection-free graft survival time in group with less than 198 pg/mL of CCL2 as compared to those with expression levels of more than 198 pg/mL (30 weeks vs. 3 weeks; log-rank test, P < 0.001). CONCLUSION: In our study, noninvasive investigation of CCL2 levels in urine has showed potential to predict rejection episodes. It is suggested that the CCL2, with others markers, may help in early detection and monitoring of graft rejection episodes.

Unique molecular alteration patterns in von Hippel-Lindau (VHL) gene in a cohort of sporadic renal cell carcinoma patients from Pakistan.

BACKGROUND: Renal cell carcinoma (RCC) is the most frequent form of kidney cancer in adults. Somatic mutations that inactivate the von Hippel-Lindau (VHL) gene are the most common cause of RCC. The frequencies of molecular changes in the VHL gene in RCCs vary among different populations. So far, a single chromosomal-based study has been reported from a South Asian population. This report presents, for the first time, the somatic changes and promoter hypermethylation in VHL in a cohort of 300 RCC patients from Pakistan. METHODS: To identify mutations in the VHL gene, direct DNA sequencing was carried out. Epigenetic silencing was investigated by using methylation-specific polymerase chain reaction. RESULTS: Our data showed molecular alterations in the VHL gene in 163 (54%) renal cell carcinoma patients. Somatic mutations were found in 87 (29%) patients and 35 novel mutations were identified. VHL promoter hyper-methylation analysis showed epigenetic changes in 106 (35%) out of 300 patients. Patients who had no evidence of molecular alterations in the VHL gene were significantly younger than patients who carried some molecular change. Molecular alterations in the VHL gene were not restricted to clear-cell RCCs (ccRCCs). CONCLUSIONS: This is the first report that identifies molecular aberrations in the VHL gene from a South Asian population. The frequency of somatic mutation is lower and that of promoter hypermethylation is higher when compared with data from other parts of the world. The data has important implications in the population-specific application of tailored preventive and therapeutic regimens in non-familial RCCs.

Frequency of complications in image guided percutaneous nephrostomy.

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.

Experience at a stricture clinic in a developing country.

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.

A kidney transplantation model in a low-resource country: an experience from Pakistan.

Pakistan is a low-resource country with a population of 185 million where expenditure on health is 1.3% of the gross national product. The estimated incidence of end-stage renal disease (ESRD) is 100 per million of the population. The paucity and high costs of renal replacement therapy render more than 90% of the ESRD population disenfranchised from replacement therapy. Our center, which is a government sector organization, established as an integrated dialysis and living related renal transplant program in the 1980s, where all services were provided free of cost to all patients with life-long follow-up care including medications. The model was based on a concept of community/government partnership where the contributions to funds vary between 40% and 60% for each partner. The model has been self sustaining for 25 years, with an annual budget of $28 million in 2010. Presently, over 600 patients are dialyzed each day and each week, 7-10 patients have received live related transplants. The overall 1- and 5-year graft survival rate of 3150 transplants is 92% and 85%, respectively. Free dialysis and transplantation established our institute as a focus of transplantation in the country. This model therefore allowed the institute to have a vital role in the campaign against transplant tourism and in the promulgation of the transplant law. It shows that in low-resource countries, specialized centers in the government sector can, with community support, provide high-quality ESRD care to the disenfranchised population.

The effect of chemokine receptor gene polymorphisms (CCR2V64I, CCR5-59029G>A and CCR5Δ32) on renal allograft survival in Pakistani transplant patients.

BACKGROUND: Gene polymorphisms of the chemokine receptors CCR2 and CCR5 (CCR2V64I, CCR5-59029G>A and CCR5Δ32) have been shown to be associated with renal allograft rejection. The aim of this study was to investigate the association of these polymorphisms with allograft rejection among Pakistani transplant patients. METHOD: A total of 606 renal transplant patients and an equal number of their donors were included in this study. DNA samples were used to amplify polymorphic regions of CCR2V64I, CCR5-59029G>A and CCR5Δ32 by polymerase chain reaction using sequence specific primers. The amplified products of CCRV64I and CCR5-59029G>A were digested with restriction enzymes (BsaB1 and Bsp12861) respectively. The CCR5Δ32 genotypes were determined by sizing the PCR amplicons. The association of these polymorphisms with the biopsy proven rejection and other clinical parameters was evaluated using the statistical software SPSS v.17. RESULTS: In this study, the G/G genotype of CCR2V64I was associated with a high frequency of allograft rejection (p=0.009; OR=2.14; 95% CI=1.2-3.7). Rejection episode(s) in the GA+AA genotypes were found to be significantly lower as compared to the GG genotype (p=0.009; OR=0.4; 95% CI=0.2-0.8). The Kaplan-Meier curve also indicated a reduced overall allograft survival for patients with the G/G genotype of CCR2V64I (59.2 ± 1.4 weeks, log p=0.008). There was a significant association with rejection by female donors possessing the CCR2 GG genotype (p=0.02; OR=2.6; CI=1.1-6.3) and male donors with the CCR5-59029 GG genotype (p=0.004; OR=1.7; CI=1.03-3.01). CONCLUSION: This study shows an association of the CCR2V64I (G/G) genotype with renal allograft rejection. However, no such association was found for the CCR5 gene polymorphisms. Therapeutic interventions such as blocking the CCR2 receptor (especially G polymorphism) may yield better survival of renal allograft in this patient group. Further, chemokine receptors may be added to the spectrum of the immunogenetic factors that are known to be associated with renal allograft rejection.

A spectrum of novel NPHS1 and NPHS2 gene mutations in pediatric nephrotic syndrome patients from Pakistan.

BACKGROUND: Mutations in the NPHS1 and NPHS2 genes are among the main causes of early-onset and familial steroid resistant nephrotic syndrome respectively. This study was carried out to assess the frequencies of mutations in these two genes in a cohort of Pakistani pediatric NS patients. METHODS: Mutation analysis was carried out by direct sequencing of the NPHS1 and NPHS2 genes in 145 nephrotic syndrome (NS) patients. This cohort included 36 samples of congenital or infantile onset NS cases and 39 samples of familial cases obtained from 30 families. RESULTS: A total of 7 homozygous (6 novel) mutations were found in the NPHS1 gene and 4 homozygous mutations in the NPHS2 gene. All mutations in the NPHS1 gene were found in the early onset cases. Of these, one patient has a family history of NS. Homozygous p.R229Q mutation in the NPHS2 gene was found in two children with childhood-onset NS. CONCLUSIONS: Our results show a low prevalence of disease causing mutations in the NPHS1 (22% early onset, 5.5% overall) and NPHS2 (3.3% early onset and 3.4% overall) genes in the Pakistani NS children as compared to the European populations. In contrast to the high frequency of the NPHS2 gene mutations reported for familial SRNS in Europe, no mutation was found in the familial Pakistani cases. To our knowledge, this is the first comprehensive screening of the NPHS1 and NPHS2 gene mutations in sporadic and familial NS cases from South Asia.

Association of the ACE-II genotype with the risk of nephrotic syndrome in Pakistani children.

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.

Polymorphisms in the methylene tetrahydrofolate reductase gene and their unique combinations are associated with an increased susceptibility to the renal cancers.

AIMS: Two single nucleotide polymorphisms in the methylene tetrahydrofolate reductase (MTHFR) gene, 677C/T and 1298A/C, encode the thermolabile isoforms of the MTHFR enzyme that adversely affect the folic acid metabolic pathway. In the present study, these polymorphisms were investigated for their associations with the risk and prognosis of the renal cell carcinomas (RCCs) in Pakistani patients. RESULTS: The study included 168 RCC patients and 178 controls. The polymorphisms were analyzed by the polymerase chain reaction-restriction fragment length polymorphism method. Statistical analysis revealed that the C-allele and homozygous C genotype of the MTHFR 1298A/C polymorphism were significantly correlated with the risk of RCCs (odds ratio [OR]=1.60; 95% confidence interval [CI]=1.1-2.34 and OR=3.26; 95% CI=1.27-8.37, respectively). The combined genotype analysis showed that the 677CC+1298CC combination greatly increased the susceptibility to RCCs (OR=8.34; 95% CI=2.7-25.7). The 677CT+1298AA and 677CC+1298CA combinations were also associated with an increased risk of RCC (OR=3.21; 95% CI=1.3-7.8 and OR=2.45; 95% CI=1.3-4.6, respectively). The combined genotype effects were also evident in a semiparametric expectation-maximization-based haplotype analysis. CONCLUSION: The results presented here indicate that the two MTHFR gene polymorphisms are significantly associated with the risk of RCCs in a cohort of Pakistani patients and may be useful as susceptibility markers in other populations of the world as well.

Association of a single-nucleotide polymorphism in the promoter region of the VEGF gene with the risk of renal cell carcinoma.

AIMS: Vascular endothelial growth factor (VEGF) protein plays an important role in tumor development and progression. Polymorphisms in the VEGF gene may lead to over- or underexpression of the protein and may be associated with either risk or progression of malignancy. The aim of this case-control study is to identify and quantify the correlation between VEGF polymorphisms and renal cell carcinoma (RCC). RESULTS: Restriction fragment length polymorphism methods were used for the analysis of VEGF polymorphisms at -2578 and +936 positions in the promoter and 3'-untranslated regions, respectively. The VEGF -2578 A-allele was associated with an increased risk of RCC (odds ratio: 1.6; 95% CI: 1.2-2.3) and A-carrier genotypes were strongly correlated (odds ratio: 2.7; 95% CI: 1.5-4.7) with higher risk. Comparison of VEGF +936 C/T polymorphism between patient and control groups revealed no association with renal carcinoma. Both VEGF -2578 C/A and VEGF +936 C/T polymorphisms showed no significant association with the histopathological parameters of RCC. CONCLUSIONS: This study shows that VEGF -2578 A-allele and A-carrier genotypes are associated with an increased risk of RCC. In groups with higher incidence of RCC, a screening test for this polymorphism may be recommended in conjunction with other established markers.

Evaluation of pre transplant T-cell activation status by soluble CD 30 determination.

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.

The outcome of extracorporeal shockwave lithotripsy for renal pelvic stone with and without JJ stent--a comparative study.

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.

Commercial transplants in local Pakistanis from vended kidneys: a socio-economic and outcome study.

Donor shortage and absence of transplant law lead to unrelated commercial transplants in Pakistan. We report the socio-economic and outcome parameters of 126 local recipients of unrelated kidney vendor transplants presenting to our institute between 1997 and 2007. Their outcome was compared with 180 recipients of living-related donor transplants matched for age, gender and transplant duration as controls. Age of commercial recipients was 35.63 +/- 11.57 years with an M:F ratio of 2.4:1. Majority (92%) were transplanted in northern Pakistan paying US$7271 +/- 2198. All were educated with 50% being graduates or above and rich earning a monthly salary of US$517 +/- 518 with 44% earning >US$500. Comparison of commercial recipients with controls showed high comorbidities 35 (28%) vs. 14 (8%) (P = 0.0001) with diabetes, hepatitis-C and cardiovascular diseases. Donor age was 29.97 +/- 6.16 vs. 32.63 +/- 9.3 years (P = 0.035). Biologic agents induction in 101 (80%) vs. 14 (8%) (P = 0.0001), acute rejections in 42 (33%) vs. 31 (17%) (P = 0.005), 1-year creatinine 1.84 +/- 1.28 vs. 1.27 +/- 0.4 mg/dl (P = 0.0001), surgical complications 28 (22%) vs. 14 (8%) (P = 0.001), tuberculosis 14 (11%) vs. 6 (6%) (P = 0.007), acute hepatitis 20 (16%) vs. 3 (2%) (P = 0.0001), cytomegalovirus 33 (26%) vs. 21 (11%) (P = 0.001) and recurrent urinary tract infection 35 (28%) vs. 30 (16%) (P = 0.034). Overall 1- and 5-year graft survival was 86% and 45% vs. 94% and 80%, respectively (P = 0.00001). Total deaths were 34 (27%) vs. 12 (6.0%) (P = 0.001). In conclusion, recipients of the vended kidneys are poor candidates, educated, rich and often self-selecting. Their outcome is poor, which will leave them poorer still and back to dialysis if not death.

Management of stone disease: 17 years experience of a stone clinic in a developing country.

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.

Treatment of ureteric calculi--use of Holmium: YAG laser lithotripsy versus pneumatic lithoclast.

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.

Comparison of holmium: Yag laser and pneumatic lithoclast in percutaneous nephrolithotomy.

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.

Celiac disease in children with persistent diarrhea and failure to thrive.

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.