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Saudi Journal of Kidney Diseases and Transplantation
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   2013| January-February  | Volume 24 | Issue 1  
    Online since January 22, 2013

 
 
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ORIGINAL ARTICLES
Single-center experience on renal transplantation in primary focal and segmental glomerulosclerosis using hematopoietic stem cell transplantation in thymus, bone marrow, portal and peripheral circulation
Aruna V Vanikar, Hargovind L Trivedi, Pankaj R Shah, Kamal V Kanodia, Rashmi D Patel, Pranjal R Modi, Shruti D Dave, Atin M Singhai, Veena R Shah, Varsha B Trivedi, V Shankar
January-February 2013, 24(1):15-21
DOI:10.4103/1319-2442.106232  PMID:23354186
Recurrence of primary focal segmental glomerulosclerosis (FSGS) is an important cause of graft loss after renal transplantation (RTx). We report our experience in 34 patients with primary FSGS who underwent RTx between April 1999 and June 2009, using hematopoietic stem cell transplantation (HSCT). They belonged to four groups: group 1 (n = 12) received high-dose HSCT in periphery, thymus, bone-marrow, and portal circulation with low-dose non-myeloablative conditio-ning; group 2 (n = 7) was modified with HSCT without marrow/thymic infusion; and group 3 (n = 3) received HSCT and proteasome inhibitor Bortezomib replacing conditioning. Group 4 (n = 12), were controls who opted for RTx under standard triple-drug immunosuppression. Patient/donor demogra-phics were comparable in all. No recurrence was noted in group 1 with mean follow-up of 8.1 years, whereas 28.6% of group 2, 33.3% of group 3, and 36.4% of group 4 had recurrence over mean follow-up of 2.6, 1.1, and 6.5 years, respectively. Mean serum creatinine was 1.62, 1.69, 1.41, and 1.73 mg%, respectively. Rejections were noted in 41.7%, 28.6%, 0%, and 45.5% grafts, respectively. Groups 1 and 4 had 25% patient loss each, group 2 had 28.6% loss, and no loss was observed in group 3. Graft loss was noted in 33.3% in group 1, 14.3% in group 2, nil in group 3, and 16.7% in the last group. Recurrent FSGS was prevented in RTx with HSCT in thymic, marrow infusion under low-dose non-myeloablative conditioning compared to controls and Bortezomib group, thus suggesting potential role of central tolerance in FSGS.
  2 2,532 514
Effect of BMI and urinary pH on urolithiasis and its composition
Qazi Najeeb, Imran Masood, Neeru Bhaskar, Harnam Kaur, Jasbir Singh, Rajesh Pandey, KS Sodhi, Suvarna Prasad, Sheikh Ishaq, Ruhi Mahajan
January-February 2013, 24(1):60-66
DOI:10.4103/1319-2442.106243  PMID:23354193
Urolithiasis is a common urological disease predominantly affecting males. The lifetime risk of urolithiasis varies from 1% to 5% in Asia, 5% to 9% in Europe, 10% to 15% in the USA and 20% to 25% in the middle-east; lowest prevalence is reported from Greenland and Japan. Such differences have been explained on the basis of race, diet and climate factors. Furthermore, changing socio-economic conditions have generated changes in the prevalence, incidence and distribution for age, sex and type of lithiasis in terms of both the site and the chemical as well as the physical composition of the calculi. The aim of our study was to determine the association between body mass index (BMI) and urine pH in patients with urolithiasis and the influence of body size, as reflected by the BMI, on the composition. The study was conducted in the Department of Biochemistry, Maharishi Markandeshwar Institute of Medical Sciences and Research, on urolithiatic patients. The data included patient's age, sex, BMI, urine pH, serum calcium, serum uric acid, serum creatinine and stone composition. Data from 100 patients, 70 men (70%) and 30 women (30%), were analyzed, with 28 patients having normal weight, 38 patients being overweight and 34 patients being obese. The mean age of the patients was 36.58 ± 9.91 years in group I, 40.47 ± 14.48 years in group II and 37.85 ± 12.46 years in group III (P > 0.05). The stone composition was calcium oxalate (CaOx) in 66 patients, calcium phosphate (CaP) in 60 patients, uric acid (UA) in 38 patients, combined calcium oxalate and calcium phosphate in 28 patients and three stones in 10 patients. The urinary pH levels (mean ± SD) were 7.78 ± 1.49 in group I, 7.15 ± 1.11 in group II and 6.29 ± 1.14 in group III patients (P = 0.0001). Urine pH showed a stepwise decrease with increasing BMI (inverse correlation). Urine pH is inversely related to BMI among patients with urolithiasis, as is the occurrence of urate, calcium oxalate and calcium phosphate stones. Similarly, the serum creatinine increased as the BMI and number of stones increased among the study population.
  2 3,573 936
CASE REPORTS
A fatal case of prostatic abscess in a post-renal transplant recipient caused by Cladophialophora carrionii
Anupma Jyoti Kindo, S Ramalakshmi, Sidhartha Giri, Georgi Abraham
January-February 2013, 24(1):76-79
DOI:10.4103/1319-2442.106249  PMID:23354196
Fungal infection secondary to renal transplantation poses a significant threat to the life of the recipient with a high rate of morbidity and mortality. A high index of suspicion is necessary for early diagnosis of fungal infections in such patients. We herein report a fatal case of prostatic abscess in a post-renal transplant recipient.
  1 1,772 305
Acute renal failure in a patient with acute lymphoblastic leukemia: A rare cause
Alok Sharma, Ruchika Gupta, Yasir Rizvi, Shyam Rathi, Manoranjan Mahapatra, Dipankar Bhowmik, Amit K Dinda
January-February 2013, 24(1):93-96
DOI:10.4103/1319-2442.106299  PMID:23354200
A young adult was diagnosed to have acute lymphoblastic leukemia, T-cell immunophenotype and was initiated on chemotherapy. He presented with acute renal failure two days after the completion of his induction regimen. A renal biopsy showed features of necrotizing crescentic glomerulonephritis (GN). Serology for c-anti-neutrophil cytoplasmic antibody (ANCA) was positive and a final diagnosis of ANCA-associated necrotizing crescentic GN was made. Aggressive immunosuppression could not be used due to the presence of nosocomial pneumonia and the patient expired 26 days after the renal biopsy diagnosis. We report for the first time the association of acute lymphoblastic leukemia with crescentic GN and, hence, expand the list of malignancy-related ANCA-positive GN.
  1 3,357 399
Acute tubular necrosis in a patient with paroxysmal nocturnal hemoglobinuria
Eranga S Wijewickrama, Lalindra Gooneratne, Chandu De Silva, Rushika L Lanarolle
January-February 2013, 24(1):105-108
DOI:10.4103/1319-2442.106302  PMID:23354203
Acute renal failure (ARF) is a well-recognized complication of paroxysmal nocturnal hemoglobinuria (PNH). The predominant mechanism is intravascular hemolysis resulting in massive hemoglobinuria ARF. We report a case of acute tubular necrosis (ATN) developed in the absence of overwhelming evidence of intravascular hemolysis in a 21-year-old man with anemia, who was eventually diagnosed to have PNH. The patient presented with rapidly deteriorating renal functions in the background of iron deficiency anemia, which was attributed to reflux esophagitis. There was no clinical or laboratory evidence of intravascular hemolysis. Renal biopsy revealed ATN with deposition of hemosiderin in the proximal tubular epithelial cells. Diagnosis of PNH was confirmed with a positive Ham's test and flow cytometry. Our case emphasizes the need to consider ATN as a possible cause for ARF in patients suspected to have PNH even in the absence of overwhelming evidence of intravascular hemolysis.
  1 2,850 514
LETTERS TO THE EDITOR
The impact of education on nutrition on the quality of life in patients on hemodialysis: A comparative study from teaching hospitals
Ali Jafarzadeh Esfehani, Sareh Dashti
January-February 2013, 24(1):130-130
DOI:10.4103/1319-2442.106308  PMID:23354209
  1 1,856 527
The impact of thyroid dysfunction on renal function tests
Abdelmula M Abdella, Botoual Seroj Ekoon, Gad Allah Modawe
January-February 2013, 24(1):132-134
DOI:10.4103/1319-2442.106310  PMID:23354210
  1 22,568 954
Is amniotic membrane a suitable biomaterial for reconstruction of long ureteral defects?
Mehdi Salehipour, Reza Mohammadian, Shahrokh Jahanbini, Vahid Emadmarvasti, Bita Geramizadeh, Nader Tanideh
January-February 2013, 24(1):135-138
DOI:10.4103/1319-2442.106311  PMID:23354211
  1 1,832 337
ORIGINAL ARTICLES
Relationship between serum leptin levels and bone mineral density and bone metabolic markers in patients on hemodialysis
Farokhlagha Ahmadi, Sina Salari, Sima Maziar, Fateme Esfahanian, Zahra Khazaeipour, Neda Ranjbarnovin
January-February 2013, 24(1):41-47
DOI:10.4103/1319-2442.106238  PMID:23354190
Leptin is the protein product of the obesity gene, which is produced in fat tissue. It was originally thought to be involved only in the regulation of food intake and energy balance. We aimed to investigate the relationship of serum leptin levels with bone mineral density (BMD) and biochemical markers of bone turnover in patients on hemodialysis (HD). This study included 72 patients (43 males and 29 females), whose mean age was 55.1 ± 11.4 years, mean body mass index was 23.13 ± 2.75 kg/m 2 and mean duration on HD was 5 ± 3.4 years. The BMD values were calculated using dual-energy X-ray absorptiometry (DEXA) at the femoral neck and lumbar spine. Blood samples were taken for leptin, intact parathyroid hormone (I-PTH), bone alkaline phosphatase (BAP), calcium (Ca), phosphate (P) and albumin. The leptin levels were higher in females than in males (22.3 ± 19.6 vs 20.8 ± 23), but this difference was not significant. The serum leptin level had a strong positive correlation with Ca levels in the female patients (r = 0.659 and P = 0.01) and a negative correlation with albumin levels (r = -0.461 and P = 0.01). No correlation was found with age, BMI, duration on dialysis, BMD and serum levels of PTH, BAP and P for the entire patient group or either gender separately. The serum leptin level was significantly lower in females with PTH >300 pg/mL when compared with patients with PTH = 100-300 pg/mL (86 ± 85 vs 47 ± 48) (P = 0.011).Women with BAP <300 IU/L had significantly higher serum leptin than those with BAP 300-600 IU/L (P = 0.024). Women with Ca <8.5 mg/dL had significantly lower serum leptin levels compared with those with Ca levels of 8.5-10.5 mg/dL (P = 0.011). There was no significant difference between the two genders among variables such as age, BMI, duration on dialysis, serum leptin, I-PTH, Ca, P, BAP, albumin and BMD of the femoral neck and lumbar spine.
  1 2,664 718
RENAL DATA FROM THE ARAB WORLD
Living and cadaver donor transplant programs in the maghreb
Jamil Hachicha, Soumaya Yaich, Khaled Charfeddine, Mondher Masmoud, Faical Jarraya, Mahmoud Kharrat, Khawla Kammoun, Mohamed Ben Hmida, Mohamed Nabil Mhiri, Mohamed Jalel Hmida, Abdelhamid Karoui, MS Ben Ammar, Taieb Ben Abdallah, Mohamed Chebil
January-February 2013, 24(1):150-156
DOI:10.4103/1319-2442.106316  PMID:23354216
In the Maghreb, organ failure constitutes a major public health problem, especially given the increasing number of patients with chronic renal failure and the high cost of care. In this study, we attempted to seek the recommendations, through a questionnaire, of various officials related to organ transplantation as well as leaders of ethics committees and religious groups in different countries of the Maghreb. The objective was to improve the rate of organ donation and transplantation. We received 36 replies (62%) within the prescribed time limit. In our survey, 83% of the respondents felt that living donor transplantation should be promoted initially, followed gradually by measures to increase cadaver donor transplantation to achieve a target of about 30 transplants with cadaver kidney donors per million inhabitants. To expand the donor pool, 83% of the respondents proposed to expand the family circle to include the spouse and inlaws. To improve the cadaver donation activity, one should improve the organizational aspects to ensure at least 50 renal transplantations per year (100%) and provide material motivation to the treatment team proportional to the activity of organ donation and transplantation. Finally, 93% of the respondents suggested suitable moral motivation of the donors.
  1 2,402 315
Pediatric renal transplantation: Jordan's experience
Issa Hazza, Reham Al-Mardini, Ghazi Salaita
January-February 2013, 24(1):157-161
DOI:10.4103/1319-2442.106361  PMID:23354217
To evaluate our experience with pediatric renal transplantation at King Hussein Medical Center, the medical records of 71 pediatric patients who underwent a renal transplantation procedure between the years 2004 and 2010 or started follow-up at our center within one week of transplantation done elsewhere were reviewed. Over the seven-year period, 71 children under the age of 14 years who received their first renal transplant were studied. About 56% (40) were males. The mean age was 9.44 ± 2.86 years. Dysplastic kidney was the most common cause of end-stage renal failure in our group, followed by glomerulonephritis. Mothers were the donors in 39.4% of the cases, followed by fathers. Twenty-three patients (32.4%) were transplanted preemptively. The overall one-year graft survival was 96%, three-year survival was 95%, and the five-year survival was 88%. Prednisone, tacrolimus, and mycophenolate mofetil formed the main-stay of immunosuppressive agents. We have developed a successful live donor program for renal transplantation in children at King Hussein Medical Center in Amman. Although our experience is still short, the graft survival is similar to that achieved in the developed world, especially with preemptive transplant.
  1 1,977 415
Ultrasound findings in urinary shistosomaisis infection in school children in the Gezira state central Sudan
Ahmed E Elmadani, Anas O Hamdoun, Ahmed Monis, Nhashal E Karamino, Nagla Gasmelseed
January-February 2013, 24(1):162-167
DOI:10.4103/1319-2442.106362  PMID:23354218
To evaluate the ultrasound findings of urinary schistosomiasis in Quran school (Khalwas) children in Gezira State Sudan, we studied all the students from two schools. A total of 103 boys were tested for urinary schistosomiasis using the urine filtration method. Schistosoma haematobium (S. haematobium) eggs were counted. Ultrasound was performed for all the positive subjects. Seventy-three (71%) subjects were positive for S. haematobium. The mean age was 11.3 ± 2.9 years. Sixty-six (90.4%) subjects showed urinary tract abnormalities. The findings revealed the following degrees of wall thickening: 53.0% mild, 18.2% moderate and 21.2% severe. Urinary bladder polyp(s) were noted in 43.3% (single) and 40.9% (multiple) of the subjects, and calcification of the bladder wall was observed in 7.6% subjects. Ureteric dilatation was noted in 38/73 (52.0%), while hydronephrosis was detected in 19/73 (26.3%). The vast majority of urinary tract schistomiasis lesions were in the urinary bladder. Ultrasound is a useful tool for identifying the morbidity of S. haematobium in endemic areas.
  1 4,765 484
RENAL DATA FROM THE ASIA - AFRICA
Prevalence of renal disease in Nigerian children infected with the human immunodeficiency virus and on highly active anti-retroviral therapy
Nosakhare J Iduoriyekemwen, Wilson E Sadoh, Ayebo E Sadoh
January-February 2013, 24(1):172-177
DOI:10.4103/1319-2442.106364  PMID:23354220
Access to highly active anti-retroviral therapy (HAART) has improved the prognosis of Nigerian children infected with the human immunodeficiency virus (HIV); thus, more children are surviving. Long-term exposure to HAART is potentially nephrotoxic. We therefore aimed at assessing the prevalence of renal disease in Nigerian children infected with HIV, who are on HAART. In this cross-sectional study, we studied children, aged ten months to 17 years, infected with HIV, attending the pediatric HIV clinics of the University of Benin Teaching Hospital. Demographic and clinical data were obtained by parental interview as well as from the medical records. Each child's urine was tested for albumin and microalbuminuria using multi test strips and mitral test strips, respectively. The serum creatinine level of each child was also estimated and used in calculating the glomerular filtration rate (GFR). Renal disease was defined as the presence of significant proteinuria of 1+ and above on dipstick or the presence of microalbuminuria of ≥20 mg and/or GFR <60 mL/min/1.73 m 2 . Of the 99 children recruited, 60 were males and 39 were females. The mean age of the children was 6.6 ± 3.5 years. All the children were on HAART and 85% had acquired the HIV infection by vertical transmission. The overall prevalence of renal disease was 16.2%. Microalbuminuria was seen in 11 children with renal disease (11.1%); 3 of them had significant proteinuria. GFR of less than 60 mL/min/1.73 m 2 was seen in five children (5.1%) with renal disease, but none had end-stage renal disease (GFR less than 15 mL/min/1.73 m 2 ). Renal disease was found to be significantly associated with advanced stage of HIV infection (P < 0.049). Our study showed that t he prevalence of renal disease in HAART-treated Nigerian children is high and majority of them are asymptomatic of renal disease, but in the advanced stages of HIV infection.
  1 2,615 559
BRIEF COMMUNICATIONS
Long-term outcome of upper limb autogenous arteriovenous fistulas for hemodialysis access
Sammy Al-Benna, David Deardon, David Hamilton, Haussam El-Enin
January-February 2013, 24(1):109-114
DOI:10.4103/1319-2442.106303  PMID:23354204
Provision of a reliable and durable vascular access for hemodialysis continues to be a challenge for clinicians. The aim of this study was to examine the outcome of upper limb autogenous arteriovenous fistula procedures for hemodialysis and factors influencing access survival. A retrospective review was carried out on 575 patients who underwent 842 consecutive vascular access procedures done over a period of ten-years. The overall primary failure rate was 5.5%, whereas the one- and five-year cumulative access survival rates were 70.0% and 52.1%, respectively. Diabetes mellitus status significantly influenced access survival (P = 0.022). Autogenous arteriovenous fistulas are reliable procedures with access sites often available in both the forearm and the arm. Patients with diabetes mellitus have significantly worse patency rates of upper limb autogenous arteriovenous fistulae.
  - 2,516 425
Buccal mucosal graft urethroplasty in long segment anterior urethral stricture - Is it gold standard?
Suresh Kumar, Punit Bansal, Mukesh K Vijay, Arindam Dutta, Punit Tiwari, Pramod K Sharma, Amit Goel, Malay K Bera, Anup K Kundu, Avijit Hazra
January-February 2013, 24(1):115-120
DOI:10.4103/1319-2442.106304  PMID:23354205
To assess the success of dorsal onlay buccal mucosal graft (BMG) urethroplasty in long segment anterior urethral stricture extending from external meatus to bulbar urethra). We studied 40 patients with long segment anterior urethral stricture, who underwent substitution urethroplasty using dorsal onlay BMG from January 2002 to December 2007. The p atients were in the age range of 15-65 years (mean 35 years) in the LS group and 16-63 years (mean 34 years) in the non-lichen sclerosus (NLS) group. The cause of stricture was LS in 20 and NLS (inflammatory and idiopathic) in the other 20 patients. The mean stricture length was 14.5 cm (range 12-17 cm) in the LS group while it was 14.0 cm (range 12-16 cm) in the NLS group. The patients were evaluated with antegrade, retrograde urethrograms and sono-urethrograms and they were followed- up with uroflometery at three months for one year, then six- monthly for two years and then annually. The contrast studies were repeated at six-monthly intervals for one year and then annually for one year. Success was defined as normal voiding pattern without any intervention post-operatively. Median follow-up was 48 months (18-72 months) in the LS group, while it was 42 months (12-72 months) in the NLS group. Among the NLS group patients, three patients developed restricture on follow-up, while seven patients among the LS group developed restricture. We conclude that the high percentage of recurrence of strictures (35%) among the LS group renders BMG urethroplasty in long segment anterior urethral stricture an unacceptable solution, and it needs further study.
  - 4,965 547
CASE REPORTS
Metastatic renal cell carcinoma of gall bladder
Deepali Jain, Prem Chopra
January-February 2013, 24(1):100-104
DOI:10.4103/1319-2442.106301  PMID:23354202
Renal cell carcinoma (RCC) is well known for its propensity to metastasize to unusual sites. Metastasis to the gall bladder (GB) has been reported in the literature rarely. We herein report an interesting case of metastatic RCC, which presented with cholecystitis. Gall bladder is a rare site of metastasis of RCC. Polypoid lesions of the GB in patients who have a synchronous or a prior history of RCC should be considered as metastatic lesions. It needs to be differentiated from primary clear cell carcinoma of the GB with the help of immunohistochemistry.
  - 2,414 401
Emphysematous pyelonephritis in a non-diabetic patient with non-obstructed kidney: An unknown entity
Indu Bhushan Dubey, Vivek Agrawal, Bhupendra Kumar Jain, Dharmendra Prasad
January-February 2013, 24(1):97-99
DOI:10.4103/1319-2442.106300  PMID:23354201
Emphysematous pyelonephritis (EPN) is an acute necrotizing infection with evidence of gas inside the kidney, perinephric space, and/or urinary collecting system. This condition is usually encountered in an obstructed urinary system of diabetics or immunocompromised patients and carries poor prognosis. The gold standard for early diagnosis is computed tomography (CT) of the abdomen. Percutaneous/surgical drainage and urgent or delayed nephrectomy are the available treatment options. We report one case of EPN, which was diagnosed in an immunocompetent non-diabetic man with a non-obstructed urinary system.
  - 2,980 548
Deafness and blindness in a renal transplant recipient with cryptococcal meningitis
Sabra Aloui, Mohamed Gorcii, Habib Skhiri, Lamia Jaafoura, Imene Gorsane, Nasr Ben Dhia, Ameur Frih, Rached Azaeiz, Elmezri Elmayl
January-February 2013, 24(1):72-75
DOI:10.4103/1319-2442.106246  PMID:23354195
Infections are a major determinant of outcome in kidney transplantation. Opportunistic pathogens are common in kidney recipients and several organs can be affected. Central nervous system infection in transplant recipients is a medical emergency. There is limited information in the literature concerning post-transplantation cryptococcal infection. Deafness and blindness are not classic findings. We report a case of meningocerebral cryptococcosis complicated by deafness and blindness after kidney transplantation. Physicians need to consider the possibility of Cryptococcus neoformans when symptoms persist despite empiric antimicrobial therapy.
  - 2,270 403
Cytomegalovirus disease in a renal transplant recipient: the importance of pre-transplant screening of the donor and recipient
Ahmed H Mitwalli, Ahmed Nazmi, Mohammed Al Ghonaim, Faisal Shaheen, Hala Kfoury
January-February 2013, 24(1):80-85
DOI:10.4103/1319-2442.106250  PMID:23354197
A 16-year-old female patient who was born with a single kidney developed chronic kidney disease during her early childhood due to reflux nephropathy and recurrent urinary tract infection. She progressed to end-stage renal disease (ESRD) and was commenced on renal replacement therapy in the form of peritoneal dialysis in May 2011. Subsequently, she underwent living unrelated donor kidney transplantation in China. She was hospitalized soon after returning to Saudi Arabia for management of high-grade fever, shortness of breath, and deterioration of renal function, which was found to be due to cytomegalovirus (CMV) disease, proved by kidney biopsy and presence of high level of anti-CMV immunoglobulins. Allograft biopsy showed mature viral particles sized between 120 and 149 nm in the nuclei of the glomerular endothelial cells. The patient was treated with valgancyclovir and specific CMV immunoglobulin, as well as by reducing and even stopping the dose of tacrolimus and mycophenolate. Despite all these measures, her condition continued to deteriorate and she finally died. Our study emphasizes that unrelated renal transplantation, especially if unplanned and improperly prepared, is a very risky procedure that might transfer dangerous diseases and increase the morbidity and mortality of the patients. We strongly stress the need for mandatory and proper screening for CMV carrier status among donors as well as recipients prior to transplantation. Also, a recommendation is made to reject CMV-positive donors.
  - 2,443 471
Florid diabetic complications in impaired glucose tolerance
Anil P Bhatt, Ankur Gupta, Deepti Vibha, Aalok Sharma, Sandeep Mahajan
January-February 2013, 24(1):86-88
DOI:10.4103/1319-2442.106251  PMID:23354198
We report a 56-year-old gentleman who had a history of impaired fasting glucose 4 years earlier but spontaneously reverted to normoglycemia. He subsequently presented with impaired glucose tolerance and proteinuria. Detailed evaluation revealed florid complications of diabetes, including nodular glomerulosclerosis of the kidney. Such complications in pre-diabetes have rarely been reported. We need to search for them early to prevent further morbidity.
  - 1,828 303
Bladder hamartoma: A unique cause of urinary retention in a child with goldenhar syndrome
Ahmed Adam, Keshree Gayaparsad, Matthys J Engelbrecht, Evelyn M Moshokoa
January-February 2013, 24(1):89-92
DOI:10.4103/1319-2442.106296  PMID:23354199
The bladder hamartoma is an extremely rare entity. We report on its presence in a 5-year-old boy with Goldenhar syndrome. Most probably, this is the first report of a bladder hamartoma presenting with obstruction of the bladder outlet resulting in urinary retention. The obstructive lesion was resected endoscopically. This proved to be curative for the lesion, since the follow-up voiding cysto-urethrogram revealed only a negligible post-void residual volume. Although urogenital anomalies have a well-known correlation with the Goldenhar syndrome, the existence of the bladder hamartoma found in association with this syndrome, according to the best of our knowledge, has not been previously reported in the world literature. With this report being only the 11 th described case of bladder hamartoma, we highlight on the management options for this exceptional histological finding. The incidence, screening, treatment decisions and important urogenital associations of the Goldenhar syndrome are also discussed.
  - 2,411 297
LETTERS TO THE EDITOR
Author's reply
Nader Aghakhani
January-February 2013, 24(1):131-131
  - 735 149
Porphyria cutanea tarda in a hemodialysis patient with hepatitis C virus: Efficacy of treatment with multiple phlebotomies and erythropoietin
Mohamed Amine Hamzi, Ahmed Alayoud, Mohamed Asseraji, Ismail Akhmouch, Zouhair Oualim
January-February 2013, 24(1):121-123
DOI:10.4103/1319-2442.106305  PMID:23354206
  - 3,848 428
Late Latent Syphilis in two hemodialysis units
O Dahmani, S Belkhalfa, K Abi ayad, J Servonnat, JF Carod, B Draou
January-February 2013, 24(1):124-127
DOI:10.4103/1319-2442.106306  PMID:23354207
  - 2,423 324
Acute bacterial endopthalmitis following dialysis catheter-related sepsis
Rehan Ahmed Siddiqui, A Harbiss Mohya, M Kechrid, O Azhari, S Shetaia
January-February 2013, 24(1):128-129
DOI:10.4103/1319-2442.106307  PMID:23354208
  - 1,460 272
Profile and outcome of infantile nephrotic syndrome treated in a tertiary care center
Prabha Senguttuvan, Hemchand Krishna Prasad
January-February 2013, 24(1):139-140
DOI:10.4103/1319-2442.106312  PMID:23354212
  - 2,625 428
APLA in post-partum renal cortical necrosis another skeleton in the closet
Dilip Gude, Sashidhar Chennamsetty, Ratan Jha, Srinivas Rao Bopparaju
January-February 2013, 24(1):141-143
DOI:10.4103/1319-2442.106313  PMID:23354213
  - 1,927 288
A novel case of persistent bartters-like syndrome associated with gentamicin exposure
Biruh Workeneh, Wiroon Sangsiraprapha, Daniel Addison, Evan Longfield
January-February 2013, 24(1):144-146
DOI:10.4103/1319-2442.106314  PMID:23354214
  - 2,023 396
Nephropathic cystinosis in a developing country: The Egyptian experience
Neveen A Soliman, Hafez M Bazaraa, Rehab H Abdel Hamid, Nora Badawi
January-February 2013, 24(1):147-149
DOI:10.4103/1319-2442.106315  PMID:23354215
  - 1,749 392
ORIGINAL ARTICLES
Post-transplantation lymphoproliferative disorders in renal vs. simultaneous renal-pancreas allograft recipients: A survey and analysis of data from the literature
Hossein Khedmat, Saeed Taheri
January-February 2013, 24(1):1-7
DOI:10.4103/1319-2442.106230  PMID:23354184
The epidemiology and other aspects of post-transplantation lymphoproliferative disorders (PTLD) are different in different transplant populations. In this study, we sought to determine the clinical, histopathological and various other features of PTLD in recipients of pancreas-renal allografts and to compare their data with renal-only transplant patients, based on the current available literature. We conducted a comprehensive search for the available data using the Pubmed and Google scholar search engines for reports of lymphoproliferative disorders after renal and simultaneous pancreas-renal (SPR) transplantations. A total of 229 recipients of renal and pancreas-renal allografts were included in the analysis. Localizations for SPR recipients were significantly higher than renal recipients in the pancreas (P <0.0001), skin (P = 0.035), liver (P = 0.043) and bone marrow (P = 0.022). Involvement of lymph nodes was more prevalent in renal recipients (P = 0.046). The occurrence of metastasis was more common among SPR recipients (P = 0.005). Hodgkin's and Hodgkin's-like PTLD were also more prevalent among SPR transplant patients (P <0.0001). Time to development of PTLD was significantly shorter among recipients of SPR (P <0.0001). In this study of international data, we found that PTLD in SPR transplant recipients have various characteristics in their site of involvement, disease presentation time and histopathological features. However, no difference in outcome was detected in these groups of PTLD patients. Future studies with larger study populations are needed for confirming and extending our study results.
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Focal and segmental glomerulosclerosis in renal allograft recipients: A clinico-pathologic study of 37 cases
Ruchika Gupta, Alok Sharma, Pranab Jyoti Mahanta, Sanjay Kumar Agarwal, Amit Kumar Dinda
January-February 2013, 24(1):8-14
DOI:10.4103/1319-2442.106231  PMID:23354185
Focal and segmental glomerulosclerosis (FSGS) in renal allografts may occur as a recurrence of primary FSGS, as a de novo phenomenon or as a complication of calcineurin inhibitor toxicity. There are very few studies in the literature describing the clinico-pathologic characteristics of FSGS in renal allografts. To the best of our knowledge, no such study exists from the Indian subcontinent. Thirty-seven cases showing FSGS, of 426 transplant biopsies performed over a 4-year period (2006-2009), were included in this study. The pre- and post-transplant clinical data were noted. FSGS was classified as per the Columbia scheme. Appropriate statistical tests were applied. The age of the study patients ranged from 13 to 54 years, with a male preponderance. Thirty-five patients (94.6%) were diagnosed as FSGS more than 12 months after transplantation. All the patients presented with renal dysfunction (median serum creatinine 2.8 mg/dL) and detectable proteinuria at the time of diagnosis. Histologically, FSGS-NOS (70.3%) was observed as the most common subtype, followed by collapsing and perihilar varieties (13.5% each). Most of the biopsies (83.7%) showed grade-2 to -3 interstitial fibrosis and tubular atrophy. Follow-up data were available in 27 patients (73%), of whom 12 (44.4%) had graft loss with dialysis-dependent state at last follow-up. FSGS is one of the important causes of graft dysfunction, especially late in the post-transplantation period in cases of de novo FSGS. The long-term outcome of renal allografts developing this glomerular pathology is quite dismal, with a significant proportion of patients suffering graft loss.
  - 3,741 762
Rapid accelerated hemodialysis in children with end-stage renal disease: A randomized clinical trial
Mohamed Khaled El Hatw, Fatina Fadel, Ramzy El Baroudy
January-February 2013, 24(1):22-29
DOI:10.4103/1319-2442.106233  PMID:23354187
In rapid accelerated hemodialysis (R-AHD), blood partially recirculates from the venous (outflow) to the arterial (inflow) line through a recirculation line (R) to selectively increase the filter blood flow rate (BFR). R-AHD PR uses two blood pump segments at the patient segment of the arterial line and at (R). To determine the effectiveness of R-AHD with regard to increasing anticoagulation and dialysis efficiency, we studied ten children with end-stage renal disease in two stages: stage 1 with 10 routine heparin R-AHD, then 10 half-dose heparin R-AHD, then 145 routine heparin R-AHD sessions for 1 month and then routine heparin double needle hemodialysis (DNHD) for one month (control). In stage 2, we dialyzed the patients with 10 routine heparin-mixed AHD PR and DNHD sessions, then eight low-dose heparin R-AHD PR" sessions, then one of the children with 10 no-heparin R-AHD PR sessions and then 10 routine heparin DNHD sessions" (control). Signs of blood clotting and dialysis efficiency were monitored. Blood clots appeared in four out of 165 R-AHD 0 (one pump circuit) sessions but in none of the 28 R-AHD PR sessions. In stage 1, the mean urea reduction rate was 0.60, 0.60 and 0.70 for the R-AHD protocols, compared with 0.71 for the control (P >0.05). In stage 2, the arterial blood urea nitrogen was reduced by 0.66 ± 0.15 after an R-AHD PR period, compared with 0.79 ± 0.18 after a DNHD period (P = 0.059). In conclusion, R-AHD PR allowed successful low heparin and no heparin hemodialysis in children without increasing the patients' BFR. However, the technique did not increase the efficiency of dialysis.
  - 2,349 375
Ultrasound predictors of compensated liver cirrhosis in hemodialysis patients with hepatitis C
P Dzekova-Vidimliski, S Dzikova, Gj. Selim, S Gelev, L Trajceska, V Pushevski, A Sikole
January-February 2013, 24(1):30-35
DOI:10.4103/1319-2442.106235  PMID:23354188
Ultrasound examination was performed in 80 hemodialysis (HD) patients with chronic hepatitis C in order to determine the ultrasound predictors of compensated liver cirrhosis. The ultrasound score (US) was calculated from the morphological parameters (liver size, morphology, surface, echogenicity and spleen volume) and the hemodynamic parameters (portal vein diameter and portal vein mean flow velocity). The US ranged from 0 to 200, with a cut-off value of 66, for discrimination between absence and presence of liver cirrhosis. A logistic regression model with stepwise variable selection was used to determine predictors of the progression of liver disease. According to the calculated US, patients were divided into two groups. The first group consisted of 37 (46.3%) patients with US greater than 66, indicating the presence of compensated liver cirrhosis. The second group included 43 (53.7%) patients without liver cirrhosis, with US equal to or less than 66. The value of liver morphology was significantly higher, but the portal vein flow velocity was significantly lower in patients with compensated liver cirrhosis compared with those without cirrhosis. Furthermore, rounded liver surfaces and increased liver echogenicity were significantly more frequent in patients with compensated liver cirrhosis compared with the non-compensated group. Logistic regression model with stepwise discriminant analysis identified liver morphology, liver echogenicity and portal vein mean flow velocity as independent ultrasound predictors of compensated liver cirrhosis in HD patients with chronic hepatitis C. Ultrasound examination could be used for non-invasive diagnosis of compensated liver cirrhosis, with accurate estimation of the disease severity in HD patients with chronic hepatitis C.
  - 2,891 417
Mucocutaneous manifestations and nail changes in patients with end-stage renal disease on hemodialysis
Ramin Tajbakhsh, Mohammad Dehghan, Ramin Azarhoosh, Ali Nobakht Haghighi, Somayeh Sadani, Soheila Samad Zadeh, Maryam Kabootari, Mostafa Qorbani
January-February 2013, 24(1):36-40
DOI:10.4103/1319-2442.106236  PMID:23354189
Mucocutaneous manifestations are common among patients on hemodialysis (HD). This study was undertaken to determine the prevalence of mucocutaneous manifestations in patients with end-stage renal disease (ESRD) who are on HD. In this cross-sectional, descriptive and analytic study conducted in 2009, 100 patients on HD at the Five Azar Hospital in Gorgan city were randomly selected. All the patients underwent detailed examination by a dermatologist to look for lesions in the skin, hair, nail and mucous membranes; if felt necessary, biopsy was obtained from the lesions. The findings were statistically analyzed using SPSS-13 software. For evaluation of normality of distribution, Kolmogorov-Smirnov was used, for quantitative variables Mann-Whitney and T-test (abnormal distribution) were used and for qualitative variables, Chi-2 and Fisher were used. In this study, P-value less than 0.05 was considered significant. Fifty-one males and 49 females were enrolled. The mean age was 49 ± 12 years. Diabetes was the most common cause of ESRD. In 95% of the patients, at least one mucocutaneous manifestation was present. Xerosis (78.3%) was the most common lesion, followed by pruritus (39.1%), lentigo (34.8%), skin discoloration (32.6%), leukonychia (32%) and thinning of the nail bed (24%). Xerosis, scaling, lentigo, folliculitis, idiopathic guttate hypopigmentation, leukonychia and half and half nail were associated with age. A significant relationship was seen between duration on dialysis and skin discoloration and leukonychia. Clubbing had a significant association with calcium-phosphorus product (Ca × P). There was a significant association between serum ferritin level and pruritus and tinea versicolor lesions. Our study shows that mucocutaneous manifestations are common among patients with ESRD. Identification of these manifestations and their association with causative factors are useful for preventing the lesions.
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Troponin-I is not falsely elevated in asymptomatic dialysis patients
Magdi M Hussein, Jaap M Mooij, Najla Al Malki, Tarek M Demerdash, Abdullah Y Mandourah
January-February 2013, 24(1):48-53
DOI:10.4103/1319-2442.106240  PMID:23354191
Dialysis patients with troponin-I levels above the cut-off value diagnostic for acute myocardial event (AME) are sometimes labeled as having "renal cause" of elevated troponin-I. Patients with troponin-I levels above 0.0 ng/mL, but below the cut-off level for an AME, are reported to have increased risk for coronary heart disease and mortality. Single pre-dialysis blood samples were taken from 150 asymptomatic dialysis patients (on hemo- or peritoneal dialysis) for troponin-I, cardiac enzymes, C-reactive protein (CRP) and lipid parameters. Troponin-I was measured by a chemiluminescent microparticle immunoassay (CMIA), of which the cut-off value for AME was set at ≥0.4 ng/mL. Patients with troponin-I levels of 0.0 ng/mL, and those with levels between 0.1 and 0.3 ng/mL, were compared regarding their cardiovascular risk profile. None of the patients had troponin-I concentrations above the cut-off level diagnostic for an AME, with 85.3% of the patients having levels of 0.0 ng/mL. While there was no difference in the "traditional" risk factors such as age, body mass index, prevalence of diabetes mellitus, hypertension, total cholesterol and low-density lipoprotein cholesterol between patients with troponin-I levels of 0.1-0.3 ng/mL and those with levels of 0.0 ng/mL, CRP concentrations were higher in the former. In peritoneal dialysis patients, the weekly Kt/V was lower in the patients with troponin levels between 0.1 and 0.3 ng/mL. The findings should add strong support in settling the debate of whether or not in patients on dialysis, falsely elevated levels of troponin-I "commonly" occur. An increased level of CRP and lower Kt/V might add to the cardiovascular risk in patients with troponin-levels between 0.1 and 0.3 ng/mL.
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Risk factors for renal scarring in children with primary vesicoureteral reflux disease
Sevgi Mir, Pelin Ertan, Nese Ozkayin
January-February 2013, 24(1):54-59
DOI:10.4103/1319-2442.106241  PMID:23354192
To determine the incidence of renal scarring among patients with primary vesicoureteral reflux (VUR) and the possible risk factor(s), we studied 90 children (60 girls and 30 boys) with VUR followed in the Pediatric Nephrology Unit at the Ege University Hospital from 1998 to 2003. All the patients were assessed for VUR grade by voiding cystoureterography and for presence of renal scarring by (99 m) technetium dimercapto-succinic acid scintigraphy. All infants with VUR were given low-dose prophylactic antibiotics and followed-up until resolution of the reflux. Grade of reflux and number of urinary tract infection (UTI) episodes (≥3) were found to be statistically significant risk factors for renal scarring (P <0.05). However, gender, familial history and laterality of the disease were not found to be statistically significant risk factors (P >0.05). Similarly, there was no statistically significant difference of frequency of renal scarring among the different age groups (P >0.05). We conclude that recurrences of UTI and VUR severity are significant risk factors for renal scarring in children with VUR. Therefore, identification of VUR at an early age may offer the opportunity to prevent episodes of UTI and possible formation of renal scars that may result in end-stage renal failure.
  - 3,597 669
Assessing the intelligence of children with chronic kidney diseases
Mostafa Amr, Abdel Hady El-Gilany, Ashraf Bakr, Eman El Sheshtawy
January-February 2013, 24(1):67-71
DOI:10.4103/1319-2442.106244  PMID:23354194
To evaluate the level of intelligence in children with chronic kidney diseases (CKDs), 12 children with CKD stage 5 on regular hemodialysis, 12 children in the predialysis stage, and 12 as controls were assessed using the Wechsler Intelligence Scale for Children (WISC). Mean scores on the verbal, performance, and full scale IQ were significantly lower in the predialysis and dialysis children than in the healthy children, while the mean score on the arithmetic subtests was significantly lower in the predialysis children than in the dialysis group. In conclusion, a better understanding of the neurocognitive function in children with CKDs is a critical element to be ascertained early with proper assessment programs so as to design appropriate educational interventions for this handicapping illness.
  - 1,977 348
RENAL DATA FROM THE ASIA - AFRICA
Diagnostic yield of kidney biopsies performed in a suburban, satellite hospital
Che Rosle Draman, Mohd Ramli Seman, Fariz Safhan Mohd. Noor, WM Kelsom
January-February 2013, 24(1):178-183
DOI:10.4103/1319-2442.106365  PMID:23354221
Kidney biopsy is indicated to confirm the clinical diagnosis or to evaluate prognosis of a renal problem. It is a reliable and safe procedure, especially with real-time ultrasound guidance. This is a single-center, retrospective review of the biopsies performed in Hospital Tengku Ampuan Afzan, Pahang from 2000 to 2010. The demographic data, clinical parameters, and histological reports were extracted from clinic records and analyzed to determine the diagnostic adequacy of biopsy samples for both lupus and non-lupus patients. A total of 219 biopsies were performed throughout the period and only 74 were included in this review. Their mean age was 22.5 ± 10.5 years. 59.5% of the biopsies were performed on female patients. Malays comprised 79.7% (n = 59) of them, followed by Chinese (18.9%, n=14) and Indian (1.4%, n=1). About one-third of the biopsies(n = 25) were performed on patients with lupus nephritis and two-thirds (n = 49) on non-lupus nephritis patients. At the time of biopsy, their serum creatinine values were normal, serum albumin 28.4 ± 10 g/L and total cholesterol 8.9 ± 4.6 mmol/L (mean ± SD). The urine dipstick was 3+ for both proteinuria and hematuria and daily protein excretion was 3.6 ± 3.2 g. Sixty-seven specimens were considered adequate and only six (8%) were inadequate for histological interpretations. The mean number of glomeruli in the biopsy specimens was 16 ± 9.9 (range: 0-47 glomeruli). In non-lupus patients, focal segmental glomerulosclerosis was the commonest histological diagnosis (n = 15, 30.6%), followed by minimal change disease (n = 13, 26.5%) and mesangial proliferative glomerulonephritis (n = 7, 14.3%). Membranous nephropathy was diagnosed in four (8.2%) and membranoproliferative glomerulonephritis in two (4.1%) specimens. Both post-infectious glomerulonephritis and advanced glomerulosclerosis were found in one specimen each. Among the lupus nephritis patients (n = 25), 88% of them were females (P <0.05) and lupus nephritis WHO class IV was the commonest variant (n = 12, 48%) followed by WHO class III (n = 7, 28%). Membranous glomerulopathy or lupus nephritis WHO class V was found in three (12%), and two (8%) had lupus nephritis WHO class II. Serum albumin, urinalysis findings, and daily urinary protein excretion were comparable for both lupus and non-lupus patients. In conclusion, renal biopsy in our center is adequate and sufficient for histological interpretations and management of patients with renal problems.
  - 2,027 275
Pattern of glomerular diseases in a tertiary care center in south India: A prospective study
Jayakumar Jeganathan, Sushanth Kumar, Mohammed Khalid, Chakrapani Maroli
January-February 2013, 24(1):168-171
DOI:10.4103/1319-2442.106363  PMID:23354219
This prospective study was undertaken to evaluate the distribution and pattern of glomerular diseases from a clinico-pathological perspective in our hospital, which is a tertiary care center in Southern India. Seventy-five consecutive patients who underwent renal biopsy and were diagnosed to have glomerular disease over a two-year period were studied and followed-up for one year. Primary glomerular disorders were more common than secondary glomerular disorders. Minimal change disease was the most common primary glomerular disease and lupus was the most common secondary glomerular disorder. Other common primary glomerular disorders were IgA nephropathy (16%) and post-infectious glomerulonephritis (10.7%), while focal and segmental glomerulosclerosis accounted for only 5.3% of the cases. In one-third of the cases, the initial clinical diagnosis did not correlate with the final biopsy diagnosis, further emphasizing the importance of renal biopsy in glomerular disorders.
  - 1,901 404
SCOT DATA
Health indicators for renal replacement therapy in Saudi Arabia

January-February 2013, 24(1):184-190
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