Renal Autotransplantation in a Patient with Bilateral Renal Artery Stenosis Secondary to Takayasu Arteritis.

Involvement of the renal artery is common in Takayasu arteritis. We, herein, present on a patient with Takayasu arteritis causing severe renal failure and a successful auto-transplantation. This case shows that early diagnosis and immediate appropriate interventions are life-saving in patients with Takayasu arteritis. Renal auto-transplantation performed in selected cases increases dialysis-free survival.

Familial Mediterranean fever-associated diseases in children.

BACKGROUND: MEditerranean FeVer (MEFV) gene encodes for the pyrin protein and a mutated pyrin is associated with a prolonged or augmented inflammation. Hence, various diseases were reported to be associated with familial Mediterranean fever (FMF) or carriers of MEFV mutations. However, systematic evaluation of all associated diseases in children with FMF has not been done previously. AIM: The aim of this study was to investigate the frequency and type of FMF-associated diseases in children. DESIGN AND METHODS: Files of FMF patients who had been seen in two reference hospitals in Ankara, in the last two years, were retrospectively evaluated. Patients with FMF and concomitant diseases were included to the study. RESULTS: Among 600 FMF patients, 77 were found to have a concomitant disease (12.8%). Thirty patients (5%) had vasculitis; 21 (3.5%) had juvenile idiopathic artritis (JIA); 7 (1.16%) had inflammatory bowel disease (IBD) and 19 had other diseases including 5 patients with isolated sacroiliitis. Overall, 13 (2.17%) patients had sacroiliitis in our cohort. The most frequent mutation was M694V/M694V (44%) and 81% of the patients had at least one M694V mutation. Majority of the patients (74%) developed associated diseases while they were not receiving colchicine therapy. CONCLUSIONS: Certain inflammatory diseases including vasculitis, chronic arthritis and IBD were more frequently detected in patients with FMF during childhood. M694V mutation is a susceptibility factor for associated diseases. In countries where FMF is prevalent, clinicians dealing with FMF and other inflammatory diseases should be aware of these associations.

Radiation exposure of patient and surgeon in minimally invasive kidney stone surgery.

OBJECTIVE: Percutaneous nephrolithotomy (PNL) and retrograde intrarenal surgery (RIRS) are the standard treatments used in the endoscopic treatment of kidney stones depending on the location and the size of the stone. The purpose of the study was to show the radiation exposure difference between the minimally invasive techniques by synchronously measuring the amount of radiation the patients and the surgeon received in each session, which makes our study unique. MATERIALS AND METHODS: This is a prospective study which included 20 patients who underwent PNL, and 45 patients who underwent RIRS in our clinic between June 2014 and October 2014. The surgeries were assessed by dividing them into three steps: step 1: the access sheath or ureter catheter placement, step 2: lithotripsy and collection of fragments, and step 3: DJ catheter or re-entry tube insertion. RESULTS: For the PNL and RIRS groups, mean stone sizes were 30mm (range 16-60), and 12mm (range 7-35); mean fluoroscopy times were 337s (range 200-679), and 37s (range 7-351); and total radiation exposures were 142mBq (44.7 to 221), and 4.4mBq (0.2 to 30) respectively. Fluoroscopy times and radiation exposures at each step were found to be higher in the PNL group compared to the RIRS group. When assessed in itself, the fluoroscopy time and radiation exposure were stable in RIRS, and the radiation exposure was the highest in step 1 and the lowest in step 3 in PNL. When assessed for the 19 PNL patients and the 12 RIRS patients who had stone sizes≥2cm, the fluoroscopy time in step 1, and the radiation exposure in steps 1 and 2 were found to be higher in the PNL group than the RIRS group (P<0.001). CONCLUSION: Although there is need for more prospective randomized studies, RIRS appears to be a viable alternate for PNL because it has short fluoroscopy time and the radiation exposure is low in every step. LEVEL OF EVIDENCE: 4.

Primary Hyperoxaluria Type 1: A Cause for Infantile Renal Failure and Massive Nephrocalcinosis.

Primary hyperoxaluria type 1 is a rare autosomal-recessive disease caused by the deficient activity of the liver specific enzyme alanine-glyoxylate aminotransferase. Increased endogenous oxalate production induces severe hyperoxaluria, recurrent urolithiasis, progressive nephrocalcinosis and renal failure. Here we report a 6 month old boy who presented with vomiting and decreased urine volume. He was diagnosed with chronic kidney failure at 4 months of age and peritoneal dialysis was introduced at a local hospital. His parents were third degree cousins and family history revealed 2 maternal cousins who developed end stage renal disease during childhood. When he was admitted to our hospital, laboratory studies were consistent with end stage renal disease, ultrasound showed bilateral massive nephrocalcinosis. As clinical presentation was suggestive for primary hyperoxaluria type 1, plasma oxalate was determined and found extremely elevated. Genetic testing proved diagnosis by showing a disease causing homozygous mutation (AGXT-gene: c.971_972delT). The patient was put on pyridoxine treatment and aggressive dialysis programme. In conclusion; progressive renal failure in infancy with massive nephrocalcinosis, especially if accompanied by consanguinity and family history, should always raise the suspicion of PH type 1. Increased awareness of the disease would help physicians in both treating the patients and guiding the families who have diseased children and plan to have further pregnancies.

Pediatric renal transplantation: a single center experience.

Renal transplantation is the treatment of choice for children with end-stage renal disease. The aim of this study was to evaluate retrospectively of our 37 pediatric renal allograft recipients, including 20 boys and 17 girls from July 2007 to August 2012. The overall mean age at transplantation was 12.16 ± 4.25 years. Three patients (8.1%) were transplanted preemptively; two were ABO-incompatible transplantations. The majority of recipients received living donor grafts (81%). The mean duration of follow-up was 25.10 ± 14.95 months. Seven acute rejection episodes were observed in 6 patients (16.2%). Eleven recipients developed serious viral infections: cytomegalovirus (n = 8), parvovirus (n = 2), BK virus (polyoma hominis 1) (n = 2), or Ebstein-Barr virus (n = 1). Three patients died; one from posttransplant lymphoproliferative disease, one from primary disease recurrence with infection, and one from sepsis. In conclusion, kidney transplantation is the treatment of choice for end-stage renal disease. Infection was the major concern after this procedure.

Anti-inflammatory and antioxidant activity of thymoquinone in a rat model of acute bacterial prostatitis.

Prostatitis plays a major role in morbidity and mortality related to prostate diseases. The aim of this study was to detect whether thymoquinone (TQ) could ameliorate oxidative damage and the proliferative response induced by Escherichia coli (E. coli) in rats. A total of 42 adult male Wistar rats were used. The rats were randomly divided into seven groups (three treatment groups, three infected groups and one control group). Control group received saline and was killed 24 h after saline administration. Infected rats were killed after 24, 48 and 72 h following direct injection of E. coli into the prostate. Treatment groups were administered with 10 mg/kg dose of TQ intraperitoneally following E. coli injection and after 24 and 48 h following E. coli injection. The rats were killed at 24, 48 and 72 h after the first drug administration. Each group was compared with each other and with the control group. In addition, infected groups were compared with treatment groups. Our findings show that the treatment with TQ has a protective effect against bacterial prostatitis-induced tissue injury. Increase in malondialdehyde levels and histological damage caused by E. coli were improved markedly with TQ treatment. TQ treatment particularly increased the activity of glutathione peroxidase and decreased the activities of catalase and superoxide dismutase. These observations might be attributed, at least in part, to the antioxidant effect of TQ and suggest that it could be a clinically valuable agent in the prevention of acute prostatitis caused by E. coli.

The effect of ankaferd to stop bleeding in experimental partial nephrectomy.

AIMS: In kidney surgery, bleeding is one of the most important issues. In partial nephrectomy, as a "blood stopper", we used surgycell and ankaferd which is used traditionally in Turkish medicine. MATERIAL AND METHODS: 24 Wistar-Albino rats were grouped randomly. Laparotomy was performed in the first group, sham group. In the second group, partial nephrectomy was performed to lower-kidney pole and then, serum physiology was given to the lower part of the kidney. In the third group, partial nephrectomy was performed and surgycell was given over the kidney. In the fourth group, partial nephrectomy was performed and then ABS was applied. In all groups, the gauze was weighted on sensitive lift before and after the operation in order to determine the amount of bleeding. After the subjects were left alive for 5 hours, the levels of blood urea, and creatinine and kidney histopathology were evaluated. RESULTS: No meaningful difference between the groups was found as for the levels of blood urea, and creatinine and the kidney histopathology. Bleeding amount was diminished significantly in the group 4, to which ankaferd was applied. CONCLUSION: Ankaferd is a substance which can be used effectively for controlling acute bleeding in kidney surgery (Tab. 2, Ref. 19). Full Text in free PDF www.bmj.sk.

The value of the levels of acute phase reactants for the prediction of familial Mediterranean fever associated amyloidosis: a case control study.

In order to determine the role of levels of acute phase proteins (APPs) for the development of amyloidosis in familial Mediterranean fever (FMF) patients, the levels of serum amyloid A (SAA), C reactive protein (CRP), fibrinogen and erythrocyte sedimentation rate were measured in paired sera of 36 FMF patients during and in between acute attacks, 39 of their healthy parents (obligate heterozgotes), and 15 patients with FMF associated amyloidosis. To compare the levels of APPs, 39 patients with chronic infections or inflammatory diseases who may develop secondary amyloidosis, 20 patients with acute infections who are known to have elevated acute phase response but will never develop amyloidosis and 19 healthy controls were included. The median levels of all APPs are increased in the patients with FMF during attacks and a significant decrease was observed after the attack was over. The level of SAA was above reference range in all FMF patients during the attack free period and the level of at least one other APP was also above normal in 64% of the patients. Both CRP and SAA levels were found to be higher in obligate heterozygotes compared to controls. The levels of SAA in patients with FMF during the attack-free period, obligate heterozygotes and patients with FMF-amyloidosis were found to be similar. The levels in each group were found to be higher than SAA levels found in healthy controls yet lower than the levels measured in the patients with acute infections and patients with chronic inflammation or chronic infections. In conclusion, our results show that SAA level reflects subclinical inflammation with high sensitivity but its value for the prediction of amyloid formation process seems to be low.

Are carriers for MEFV mutations "healthy"?

OBJECTIVE: We aimed to compare whether carriers for the MEFV mutations display an increase or decrease in certain features. We compared the frequency of a number of inflammatory symptoms and diseases in carriers and a control population. METHODS: A questionnaire was designed to be applied to parents of children with FMF and a control group of parents. Clinical features and some diseases including the frequency of febrile episodes, abdominal pain, arthralgia, prophylaxis with penicillin, acute rheumatic fever, rheumatoid arthritis, vasculitis, spondyloarthropathy, urinary tract infection, asthma, allergy, irritable bowel disease, appendectomy and tonsillectomy were inquired. 676 parents of 440 children with FMF were surveyed in this study. Controls (n: 774) were selected as parents of healthy children. RESULTS: The presence of febrile episodes more than four per year, arthralgia, past diagnosis for acute rheumatic fever, rheumatoid arthritis and prophylaxis of penicillin, acute rheumatic fever, and rheumatoid arthritis were significantly higher in asymptomatic parents for the MEFV mutations compared to controls. The frequency of allergy was found to be significantly lower in the asymptomatic parents as compared to controls. There was no significant difference at the frequency of urinary tract infection and tonsillectomy between the parents of the patents and controls. CONCLUSIONS: We suggest that one MEFV mutation may indeed be conferring a heightened inflammation as suggested by the increased frequency in inflammatory symptoms. The carrier status for MEFV mutations seem to be unique, in that they cause an alteration in the state of "health".

The "nutcracker phenomenon" with orthostatic proteinuria: case reports.

The nutcracker phenomenon refers to compression of the left renal vein between the aorta and the superior mesenteric artery. Clinical features are hematuria, abdominal pain, left flank pain, pelvic or scrotal discomfort due to varicocele or ovarian vein syndrome. In this report, 2 patients with orthostatic proteinuria, in whom nutcracker phenomenon was detected as a cause, are presented. One of them had posterior nutcracker with also asymptomatic varicocele that was detected during ultrasonographic examination. Nutcracker phenomenon is a rare but important clinical condition that should be considered in the differential diagnosis of patients with proteinuria and hematuria.

Pulmonary involvement in childhood-onset systemic lupus erythematosus: a report of five cases.

OBJECTIVE: Systemic lupus erythematosus (SLE) is a chronic systemic disease, which can involve multiple organs such as kidney, skin and brain. Lung is another organ that can be affected. A number of pulmonary complications including pleuritis, pneumonitis, infectious pneumonia, pulmonary haemorrhage, pulmonary hypertension and pneumothorax have been reported in patients with SLE. Pulmonary involvement is relatively frequent in adult patients; it has infrequently been reported in children with SLE. However, pulmonary manifestations may be an initial and/or life-threatening complication of SLE in children. In this paper we aim to emphasize the pulmonary involvement in childhood-onset SLE via description of our patients. METHODS: The patients, who were diagnosed with SLE at the Children's Hospital of Ankara University Medical School between 1993 and 2002, were retrospectively evaluated for evidence of pulmonary involvement. All patients fulfilled at least four of the classification criteria of the American Rheumatism Association. Using a standardized form, we obtained data regarding the age, sex and presenting complaints of the patients, previous therapies given, clinical and laboratory features, treatment and outcome. Informed consent was obtained from all patients. RESULTS: During the 10-yr study period, 16 patients were diagnosed with childhood-onset SLE. Five of them (31%) had pulmonary involvement including acute lupus pneumonitis, invasive pulmonary aspergillosis, cytomegalovirus pneumonia and pulmonary haemorrhage (in two patients). These 5 patients with lupus lung disease are presented in more detail.

Is dorsale penile vein ligation (dpvl) still a treatment option in veno-occlusive dysfunction?

PURPOSE: To determine the long-term results of the DPVL for the treatment of venous impotence. PATIENTS AND METHODS: The long term results of DPVL in 134 patients were interviewed. The mean age was 39.2 (range 21-72). Power color doppler imaging, pharmacocavernography/dynamic cavernosometry were performed. Postoperatively, all the patients were controlled in the 6th month, first year and, if possible, once a year. The mean follow-up was 54.8 (14-76) months. Postoperative outcomes were classified into three groups: complete spontaneous erection (CR), response to pharmacotherapy (PR) or no satisfactory improvement (NR). RESULTS: The short-term success in the 6th month according to above mentioned was 38.8%, 18.6% and 42.5%; and in the first year was 19.4%, 14.9% and 65.6% respectively. The result in 35 patients whose follow-up was at least 5 years (mean 67 months) was 11.4%, 14.3% and 74.3% respectively. Positive prognostic factors were preoperative age <40, duration of erectile dysfunction <2 years, non-smoker patients, non neurogenic disease and distal disease. With all these parameters present, long-term success (CR, PR) rose from 33.6% to 55.9% (P < 0.001). CONCLUSIONS: Long-term success for unselected patients undergoing DPVL is disappointing; however, careful patient selection significantly improves long-term results.

Colchicine treatment in children with familial Mediterranean fever.

Prophylactic colchicine therapy has been shown to be a safe and effective method of eliminating the attacks and preventing the development of amyloidosis in patients with familial Mediterranean fever (FMF). However, information about effective dosages that control FMF attacks and prevent amyloidosis in childhood is not available. The aim of this study is to determine the 'effective colchicine dose' for children in terms of body weight and surface area. Sixty-two (34 male, 28 female) children with FMF were selected and colchicine treatment was initiated by giving 0.5-1 mg/day to each patient. The dose was gradually increased up to a maximum 2 mg/day in unresponsive patients; mean duration of therapy was 45.6 +/- 35.5 months. When the 'optimal effective dosage' (i.e. the one that reduced the frequency of attacks and ESR, CRP and fibrinogen levels during the attack-free period) was achieved, the optimal effective dose was calculated according to the body weight and body surface area for each patient. Based on these values 'mean colchicine dose' was computed for the study group and values for different age groups were evaluated. Mean colchicine doses according to the body weight and surface area of the whole group were found to be 0.03 +/- 0.02 mg/kg/day and 1.16 +/- 0.45 mg/m(2)/day, respectively. It was shown that children less than 5 years of age might need colchicine doses as high as 0.07 mg/kg/day or 1.9 mg/m(2)/day. These dosages are approximately 2.5-3 times more than the 'mean colchicine dose' for children aged 16-20 years. These results clearly show that small children need higher doses of colchicine in order to control their attacks. Thus, we conclude that colchicine, when given according to body weight or body surface area, would be more effective in childhood.

Is dorsale penile vein ligation (DPVL) still a treatment option in veno-occlusive dysfunction?

PURPOSE: To determine the long-term results of the DPVL for the treatment of venous impotence. PATIENTS AND METHODS: The long-term results of DPVL in 134 patients were interviewed. The mean age was 39.2 (range 21-72). Power color doppler imaging, pharmacocavernography/dynamic cavernosometry were performed. Postoperatively, all the patients were controlled in the 6th month, first year and, if possible, once a year. The mean follow-up was 54.8 (14-76) months. Postoperative outcomes were classified into 3 groups: complete spontaneous erection (CR), response to pharmacotherapy (PR) or no satisfactory improvement (NR). RESULTS: The short-term success in the 6th month according to above mentioned was 38.8%, 18.6% and 42.5%; and in the first year was 19.4%, 14.9% and 65.6% respectively. The result in 35 patients whose follow-up was at least 5 years (mean 67 months) was 11.4%, 14.3% and 74.3% respectively. Positive prognostic factors were preoperative age <40, duration of erectile dysfunction <2 y, non-smoker patients, non neurogenic disease and distal disease. With all these parameters present, long-term success (CR, PR) rose from 33.6% to 55.9% (p < 0.001). CONCLUSIONS: Long-term success for unselected patients undergoing DPVL is disappointing; however, careful patient selection significantly improves long-term results.

Risk factors for penile prosthetic infection.

OBJECTIVES: To determine the risk factors for penile prosthesis infection. METHODS: The records of 135 penile prosthesis implantation in 127 patients were reviewed. Of the 135 prothesis, 115 were malleable, 12 were self-contained and 8 were inflatable. Of these procedures, 111 were primary, 9 were primary with reconstructions and 15 were secondary. Mean follow-up was 47 months (minimum 6 months). All of the reconstructions were penile plications or plaque excisions for Peyronie's disease. RESULTS: The ratio of penile prosthesis infection was 8.89%. Secondary implantation, paraplegia, non-controlled diabetes mellitus (p < 0.001) and surgeon's inexperience (p < 0.05) were detected as the risk factors for penile prosthesis infection. But age, smoking, alcohol consumption, obesity, atherosclerosis, presence of diabetes mellitus (DM), history of penile surgery, simple penile reconstruction, type of the erectile dysfunction (ED), type of the penile prostheses and incision and were not found as the risk factors (p > 0.05). CONCLUSIONS: Paraplegie, non-controlled diabetes mellitus, secondary implantation and surgeon's inexperience appear to be the risk factors for penile prosthesis infection. In secondary implantation, longer operation time is detected as a factor increasing the risk of penile prosthesis infection. For these patients, careful preoperative preparation, more attention to perioperative antisepsis and postoperative follow-up are required. Since it has been determined that surgical experiences decrease the complication rate, these patients should be operated by experienced surgeons.