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Biomedical subjects

S Conrad

Publications and source records attributed to S Conrad.

At least 19 recordsLinked to original sources
PubMed2001

Evaluation of a vincristine resistant Caco-2 cell line for use in a calcein AM extrusion screening assay for P-glycoprotein interaction.

AIM: To develop a fast fluorometric screening assay based on vincristine resistant Caco-2 cells (Caco-2VCR) in order to elucidate potential P-glycoprotein (Pgp) interactions of compounds, and to characterise Caco-2VCR cells with regard to their expression of the efflux transporters Pgp, MRP1 and MRP2. METHODS: We applied the Caco-2VCR cells to a 96-well plate-based calcein AM extrusion assay. The Caco-2VCR cells were cultured as monolayers and incubated with calcein AM with/without addition of Pgp modulators. Fourteen known Pgp modulators were tested in the assay (chloropromazine, cyclosporin A, domperidone, digoxin, ivermectin, ketoconazole, loperamide, metoprolol, propranolol, progesterone, quinidine, quinine, verapamil and vincristine). For each compound an EC50 value was calculated. Protein and mRNA levels of the efflux transporters were analysed by Western blot and polymerase chain reaction techniques. RESULTS: All compounds with the exception of digoxin displayed increased calcein levels. Protein and mRNA analysis showed increased levels of Pgp after vincristine exposure, while expression of the efflux transporters MRP1 and MRP2 remained unchanged. CONCLUSIONS: The calcein AM extrusion assay applied to Caco-2VCR cells can be a valuable tool as a screening assay for new compounds and their potential interaction with P-glycoprotein.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed2000

[Prognostic markers for prostate cancer].

Careful consideration of therapy for prostate cancer needs an accurate analysis of prognostic markers to estimate success and benefit for the patient. Prognosis of prostate cancer is determined by the proportion of high grade cancer, many usually utilized prognostic characteristics were rejected by multivariate analysis as no independent prognostic information was delivered. For planning therapy it is crucial to estimate the proportion of high grade cancer as exact as possible. A standardized biopsy technique combined with a quantified analysis of the biopsy cores is most helpful to achieve this goal. The prognostic value of molecular biological and other factors is object of research, only a combination of some of these factors could be demonstrated so far to be superior to the estimation of the proportion of high grade cancer. However, at present their routine use in daily practise is precluded by a high technical and financial expense.

Biomarkers, Tumor↗
PubMed2000

Short- and medium-term results of the thrust plate prosthesis in patients with polyarthritis.

The thrust plate prosthesis is an implant with metaphyseal fixation to the proximal femur, which leaves the diaphyseal bone untouched. Therefore, this implant is preferred in younger patients. It is dependent on good bone quality in the proximal femur. Because the bone quality is reduced in patients with polyarthritis, this kind of endoprosthesis may have a higher failure rate than conventional stemmed endoprostheses in these patients. Therefore, in patients with polyarthritis, even short- and medium-term results of the thrust plate prosthesis should be analyzed. In all, 47 thrust plate prostheses were implanted in 42 patients with polyarthritis (29 with rheumatoid arthritis, 6 with juvenile chronic arthritis, and 7 with spondylarthritis) and followed prospectively. The average age at operation was 40.8+/-10.7 years. Each patient was clinically and radiologically examined preoperatively, 3 and 6 months after the operation, and at the end of each postoperative year. The mean follow-up was 26.1+/-10.7 months. The clinical findings were evaluated using the Harris hip score. Radiologically, 8 different zones of the thrust plate prosthesis were analyzed for radiolucencies. During the 1st year, the Harris hip score rose continuously from the preoperative average of 42.4+/-6.5 points to 78.8+/-10.3 points 3 months postoperatively, 82.3+/-9.8 points 6 months postoperatively, and 86.8+/-10.1 points 1 year after the operation. The subsequent examinations showed Harris hip score remained at the same level. Five patients (5 joints, 10.6%) had to undergo a revision of the thrust plate prosthesis due to aseptic loosening in 3 and septic loosening in 2. Six prostheses (12.6%) showed radiolucencies, mostly below the thrust plate in zones 1 and 2. Two of them were certainly radiologically loose, which raised the failure rate to 7 of 47 (14.8%). The thrust plate prosthesis improves function and alleviates pain in patients with polyarthritis to a satisfactory degree. Concerning the failure rate, this type seems to yield slightly worse results than cementless stemmed endoprostheses in the same patient group. Due to the preservation of the diaphyseal bone of the femur and the possibility of an unproblematic change to a stemmed endoprosthesis, the thrust plate prosthesis can be recommended for younger patients with polyarthritis.

Adult↗
PubMed2000

Activity of the hypothalamic-pituitary-adrenal axis is altered by aging and exposure to social stress in female rhesus monkeys.

Age-related changes in glucocorticoid negative feedback inhibition of hypothalamic CRF and pituitary ACTH are observed in rodents. Attempts to study similar effects in humans have produced mixed results due in part to the difficulty in matching older subjects on social and lifestyle variables. The present study used female rhesus monkeys as a model for women by comparing young adult (n = 20) to old (n = 20) females to test the hypotheses that the hypothalamic-pituitary-adrenal axis is altered in older animals and that this difference is exacerbated by exposure to social stress. The effects of age on the response to two doses of dexamethasone and two doses of CRF were assessed in females living in a stable social environment (control) and in socially stressed females removed from their group and housed temporarily in a remote, nonsocial environment (separated). The suppression of serum cortisol was not different between the two doses of dexamethasone. Before dexamethasone administration (2100 h), serum cortisol was significantly higher in old control females than in either young or old separated females, who were not different from one another. The young control females had baseline cortisol concentrations significantly lower than all other groups. Serum cortisol was suppressed approximately 75% below baseline values in all groups by 10 h after dexamethasone administration. Age significantly affected serum cortisol after dexamethasone, as the old control group showed a release from suppression 19 h posttreatment compared to the young control group and compared to the separated groups. Social condition had a significant effect on the responses of serum cortisol and plasma ACTH to CRF administration. At baseline (0930 h), serum cortisol was significantly higher in young controls compared with older controls, with both separated groups having intermediate values. Similarly, plasma ACTH at baseline was significantly higher in young controls compared to all other groups. Social separation significantly diminished the elevation of both serum cortisol and ACTH after stimulation with either dose of CRF. Control females showed a prolonged increase in plasma ACTH through 60 min and an increase in serum cortisol through 120 min after CRF. In contrast, these hormones either declined by 60 min or did not increase in socially separated females after CRF administration. These data suggest that the circadian rhythm in serum cortisol may be affected by aging, as levels were higher in the evening and lower in the morning in old control compared to young control females. The effect of age on the response to dexamethasone treatment among the control groups lends support to the hypothesis that the sensitivity of glucocorticoid negative feedback diminishes with aging. Although age did not affect the response to CRF, social separation diminished the elevation in both serum cortisol and plasma ACTH. Whether this effect was due to stress-induced down-regulation of pituitary CRF receptors remains to be determined.

Aging↗
PubMed2000

Detection of loss of heterozygosity in the p53 tumor-suppressor gene with PCR in the urine of patients with bladder cancer.

PURPOSE: Detection of loss of heterozygosity (LOH) has been described in various carcinomas on the basis of meticulous molecular techniques. Because of lack of simple and rapid techniques, LOH has not achieved common use in routine tumor diagnosis. A recently found variable number of tandem repeats (VNTR) segment in intron 1 of the p53 gene was described as highly polymorphic and therefore useful in detecting LOH. We used a rapid technique for detection of LOH in the p53 gene of patients with transitional cell carcinoma (TCC) of the bladder. The technique was based on the polymerase chain reaction (PCR) and agarose gel electrophoresis as described for other carcinomas previously. We evaluated whether TCC screening and surveillance could be performed detecting LOH in the urinary sediment. MATERIALS AND METHODS: We investigated 29 patients with TCC of the bladder (pTa 12 patients; pT1 10 patients; pT2 - pT4 seven patients; grade 1 one patient; grade 2 19 patients; grade 3 nine patients). DNA was prepared by standard methods from white blood cells, tumor tissue, normal bladder mucosa, and urinary sediments. The amplification of the VNTR region was performed with PCR. PCR products were run in parallel lanes on 4.5% agarose gels. RESULTS: Of the 29 patients, 23 (79.3%) were found to have two different alleles ("informative cases") for the VNTR region. Of the 23 informative cases LOH was detected in the tumor tissue of 10 patients (43.5%). Referring to the total population 10 of 29 patients (34.4%) revealed LOH. In all patients with LOH in the tumor, LOH was also detected in the urinary sediment. LOH was not detected in the histologically benign bladder mucosa. CONCLUSION: We present a simple and rapid technique based on PCR and agarose gel electrophoresis for the detection of LOH in tumor and urinary sediment of patients with TCC of the bladder. The ability to detect LOH not only in tumor tissue but also in urinary sediment offers an attractive approach for noninvasive diagnosis and surveillance of bladder cancer patients.

Carcinoma, Transitional Cell↗
PubMed2000

Detection rate of histologically insignificant prostate cancer with systematic sextant biopsies and fine needle aspiration cytology.

PURPOSE: We evaluate the detection rate of insignificant prostate cancer and the rate of significant prostate cancer overlooked in the results of systematic sextant biopsy and fine needle aspiration biopsy of the prostate of asymptomatic men with serum prostate specific antigen concentrations less than 4.0 ng./ml. MATERIALS AND METHODS: We analyzed specimens from 133 consecutive patients with a mean age of 60 years undergoing cystoprostatectomy for bladder cancer. Six systematic biopsy specimens and 2 fine needle aspiration cytology samples were taken from the prostate immediately after cystoprostatectomy. The specimens were step sectioned and examined for prostate cancer. Insignificant prostate cancer was defined as any cancer with an aggregate volume 0.5 cm.3 or less. RESULTS: Incidental prostate cancer was found in 58 of the 133 patients (44%). Tumor volume was 0.5 cm.3 or less in 47 cases. Sextant biopsy detected 7 cancers, including 4 of 47 (9%) that were insignificant and 3 of 11 (27%) that were significant. Fine needle aspiration cytology also detected 7 cancers, including 3 (6%) and 4 (36%) that were insignificant and significant, respectively. CONCLUSIONS: Systematic sextant biopsy and fine needle aspiration cytology each diagnose prostate cancer in about 5% of asymptomatic men who have normal digital rectal examination and serum prostate specific antigen less than 4.0 ng./ml. However, many of the cancers thus detected are insignificant and most of the significant cancers are missed. Therefore, routine screening of such patients with sextant biopsy or aspiration cytology does not appear to be justified.

Adenocarcinoma↗
PubMed1999

[Perioperative incidence of transfusions after allogenic kidney transplantation].

The requirement of blood transfusions was evaluated in a two compartment (retrospective/prospective) study in our renal transplantation program. Between July 1st, 1993 and December 31st, 1994 (observation period 1) we retrospectively investigated 110 patients with end stage renal disease and anemia undergoing kidney transplantation. Between January 1st, 1995 and December 31st, 1996 (observation period II) the requirement of blood transfusions was followed prospectively in 134 patients after allogenic renal transplantation. The amount of blood drawn for preoperative diagnostic investigations was in observation period 1 significantly higher (280 ml) than in observation period II (150 ml) (p = 0.02). For postoperative diagnostic tests in observation period II significantly less blood (240 ml) was needed than in observation period 1 (510 ml) (p = 0.01). The intraoperative blood loss was similar in both periods (170 ml vs. 190 ml; p = 0.6). The need for closer graft observation was the reason for significantly increased amount of blood transfusions in patients with delayed graft function. The number of blood transfusions was significant lower in patients with primary graft function (p = 0.0001). There was no correlation between blood transfusions and the use of ATG/OKT3, surgical complications and reoperations. With an improved management of blood drawing for diagnostic tests after allogenic kidney transplantation the number of perioperative blood transfusions can be reduced significantly.

Adolescent↗
PubMed1999

Early prostate-specific antigen relapse after radical retropubic prostatectomy: prediction on the basis of preoperative and postoperative tumor characteristics.

OBJECTIVES: This study was undertaken to distinguish between patients who will and will not benefit from a retropubic radical prostatectomy (RRP) for clinically localized prostatic carcinoma (PCa) on the basis of preoperative and postoperative tumor characteristics. METHODS: Data of 318 consecutive patients who underwent RRP for clinically localized PCa were reviewed. Preoperative characteristics used included clinical stage, findings on transrectal ultrasonography, prostate-specific antigen (PSA) values, Gleason grade, number of positive biopsies, number of biopsies containing any Gleason grade 4 and/or 5 cancer, and number of biopsies with predominant (>50% of cancerous tissue) Gleason grade 4 and/or 5 cancer. Postoperative characteristics included pathologic stage, Gleason grade, margin status, cancer volume, and volume of Gleason grade 4 and/or 5 cancer. The impact on biochemical relapse after RRP were calculated by Cox regression and CART (classification and regression tree) analysis to establish low, intermediate, and high risk of recurrence. RESULTS: Of patients who underwent RRP, 66% showed no evidence of relapse after a follow-up of 42 months. All preoperative and postoperative characteristics showed a significant association with biochemical relapse. Cox regression of preoperative characteristics showed the number of positive biopsies with predominant Gleason grade 4 and/or 5 cancer to be the most accurate predictor of failure (p < 0.0001), followed by the number of positive biopsies and PSA. CART analysis distinguished between four risk groups on the basis of the same characteristics as in the Cox regression. The low-risk group consisted of 232 patients (75.1%) and the high-risk group of 17 patients (5.5%); corresponding Kaplan-Meier curves showed a 2-year PSA-free survival rate of 97% for the low-risk group and 20% for the high-risk group. Cox regression of postoperative characteristics recognized the volume of Gleason grade 4 and/or 5 as the characteristic with the strongest association with biochemical failure. CART analysis distinguished between four risk groups, using the volume of high-grade cancer as the most influential characteristic. The corresponding Kaplan-Meier curves showed for the low-risk group (n = 79; 29.6%) a PSA-free survival rate of 96% after 42 months and for the high-risk group (n = 47; 17.6%) a 21% PSA-free survival rate after 42 months. CONCLUSION: For preoperative and postoperative estimation of biochemical recurrence after RRP, a quantitative analysis of high-grade cancer, expressed by the number of preoperative biopsy cores containing high-grade cancer and the volume of cancer, proved to be the best predictor of relapse. CART analysis might be useful in advising patients for their best therapy options. However, defined characteristics of risk groups should be evaluated with new prospective data before they are used routinely.

Adenocarcinoma↗
PubMed1999

The role of C-reactive protein in graft dysfunction after renal transplantation.

PURPOSE: We investigated whether serial daily measurements of serum C-reactive protein could help differentiate episodes of transplant dysfunction due to rejection, infection, cyclosporin A nephrotoxicity or acute tubular necrosis in renal allograft recipients. MATERIALS AND METHODS: Morning serum was obtained daily from 134 patients during the first 30 days after renal transplantation. All episodes of graft dysfunction were recorded and compared to transplant biopsies. C-reactive protein concentrations were correlated with postoperative graft function and the various causes of graft dysfunction. RESULTS: All patients demonstrated an increase in C-reactive protein in response to surgery and a maximum level was reached on day 2 after transplantation. Mean C-reactive protein concentration was significantly increased for delayed (61.50 microg./ml.) compared to primary (mean 38.01) graft function (p = 0.001, Mann-Whitney U test). There were significant increases in C-reactive protein for bacterial infections other than asymptomatic bacteriuria (33.98 microg./ml), interstitial graft rejection (16.43) and acute tubular necrosis (30.50) compared to uneventful courses. C-reactive protein was unchanged for viral infections or cyclosporin A toxicity. CONCLUSIONS: Serial C-reactive protein measurements help to identify renal transplant dysfunction of different origins. However, rejection, infection and acute tubular necrosis show similar patterns of C-reactive protein increase. Thus, C-reactive protein is unable to discriminate the causes of renal graft dysfunction. Biopsy remains the gold standard for the differential diagnosis of renal allograft dysfunction.

Adolescent↗
PubMed1998

Improvement of ureteroileal anastomosis in continent urinary diversion with modified Le Duc procedure.

PURPOSE: Ureteroileal stricture after urinary diversion often has deleterious effects on the upper ureteral tract. A common ureteral implantation technique described by Le Duc et al has recently been criticized for a high rate of ureteral stricture. We assessed the rate of upper urinary tract dilatation and deterioration in 60 consecutive patients who underwent continent urinary diversion and ureteroileal anastomosis with a modified Le Duc procedure. MATERIALS AND METHODS: A total of 60 patients underwent continent urinary diversion with an ileal neobladder (Hautmann). Ureteroileal implantation was performed using a modified Le Duc technique. Followup was performed prospectively for a median of 32 months. The clinical end point was postoperative development of upper urinary tract dilatation, or an increase in preoperative dilatation confirmed by excretory urography or furosemide isotope nephrography. RESULTS: Nonneoplastic deterioration of the upper urinary tract was noted in 4 of 117 renoureteral units (3.4%). No permanent decrease in renal function or increase in pyelonephritic episodes was noted. CONCLUSIONS: The modified Le Duc technique is simple and safe for ureteroileal anastomosis and has a low complication rate.

Adult↗
PubMed1998

Systematic sextant biopsies improve preoperative prediction of pelvic lymph node metastases in patients with clinically localized prostatic carcinoma.

PURPOSE: An algorithm including the results of systematic sextant biopsies was statistically developed and evaluated to predict the probability of pelvic lymph node metastases in patients with clinically localized carcinoma of the prostate. MATERIALS AND METHODS: Clinical stage, serum prostate specific antigen concentration, Gleason score, number of positive biopsies, number of biopsies containing any Gleason grade 4 or 5 cancer and number of biopsies predominated by Gleason grade 4 or 5 cancer were recorded in 345 patients undergoing pelvic lymph node dissection and correlated with the incidence of lymph node metastases. Multivariate logistic regression, and classification and regression trees analyses were performed. RESULTS: In univariate analysis all variables had a statistically significant influence on lymph node status. Logistic regression showed that the amount and distribution of undifferentiated Gleason grade 4 and 5 cancer in the biopsies were the best predictors of lymphatic spread followed by serum prostate specific antigen. Classification and regression trees analysis classified 79.9% of patients who had 3 or fewer biopsies with Gleason grade 4 or 5 cancer and no biopsies predominated by undifferentiated cancer as a low risk group. In this group positive lymph nodes occurred in only 2.2% (95% confidence interval 0.8 to 4.7%). CONCLUSIONS: Including the results of systematic sextant biopsies substantially enhances the predictive accuracy of algorithms that define the probability of lymph node metastases in prostatic cancer. Patients thus defined as having no lymphatic spread could potentially be spared pelvic lymph node dissection before definitive local treatment.

Adenocarcinoma↗
PubMed1998

Altered sphingomyelinase and ceramide expression in the setting of ischemic and nephrotoxic acute renal failure.

Diverse physical and chemical stimuli can activate sphingomyelinases (SMases), resulting in sphingomyelin (SM) hydrolysis with ceramide release. Since ceramide can profoundly impact a host of homeostatic mechanisms, the concept of a "SM (or SMase) signaling pathway" has emerged. We recently documented that ceramide levels fall abruptly during renal ischemia, and then rebound to twice normal values during early reperfusion (30 to 90 min) Therefore, the present study assessed whether these ceramide changes are paralleled, and hence potentially mediated, by comparable changes in SMase activity. Mice were subjected to 45 minutes of renal ischemia +/- 30 minutes, 90 minutes, or 24 hours of reperfusion. Renal cortices (or isolated proximal tubules) were then assayed for SMase activity (acidic, neutral forms). To characterize whether early post-ischemic ceramide increments are a relatively persistent event, ceramide was assayed following a 24-hour reperfusion period. Finally, to assess whether the observed perturbations were unique to post-ischemic injury, SMase and ceramide were quantified in the setting of glycerol-induced myohemoglobinuria and anti-glomerular basement membrane (alpha GBM) antibody-induced acute renal failure (ARF). Ischemia induced abrupt declines (approximately 50%) in both acidic and neutral SMase activities, and these persisted in an unremitting fashion throughout 24 hours of reperfusion. Nevertheless, increased ceramide expression (2x normal) resulted. Myohemoglobinuria also suppressed acidic/neutral SMases, and again, "paradoxical" ceramide increments were observed. Finally, alpha GBM nephritis increased ceramide levels, but in this instance, a correlate was increased SMase activity. These results suggest that: (1) ceramide is an acute renal "stress rectant" increasing in response to diverse renal insults; (2) this response may occur independently of the classic SM pathway, since the ceramide increments can seemingly be dissociated from increased SMase activity; and (3) given the well documented impact of ceramide and the SM(ase) pathway on apoptosis, cell proliferation, differentiation, and tissue inflammation, the present results have potentially broad ranging implications for the induction and evolution of diverse forms of ARF.

Acute Kidney Injury↗
PubMed1998

Partial liquid ventilation in adult patients with ARDS: a multicenter phase I-II trial. Adult PLV Study Group.

OBJECTIVE: To evaluate the safety and efficacy of partial liquid ventilation (PLV) in adult patients with the acute respiratory distress syndrome (ARDS). SUMMARY BACKGROUND DATA: Previous studies have evaluated gas exchange and the safety of PLV in adult patients with severe respiratory failure whose gas exchange was partially provided by extracorporeal life support (ECLS). This is the first experience with adult patients who were not on ECLS. METHODS: Intratracheal perflubron in a total dose of 30.1 +/- 7.1 ml/kg was administered over a period of 45 +/- 9 hours to nine adult patients with mean age = 49 +/- 4 years and mean PaO2/FiO2 ratio = 128 +/- 7 as part of a prospective, multicenter, phase I-II noncontrolled trial. RESULTS: Significant decreases in mean (A-a)DO2 (baseline = 430 +/- 47, 48 hour = 229 +/- 17, p = 0.0127 by ANOVA) and FiO2 (baseline = 0.82 +/- 0.08, 48 hour = 0.54 +/- 0.06, p = 0.025), along with an increase in mean SvO2 (baseline = 75 +/- 3, 48 hour = 85 +/- 2, p = 0.018 by ANOVA) were observed. No significant changes in pulmonary compliance or hemodynamic variables were noted. Seven of the nine patients in this study survived beyond 28 days after initiation of partial liquid ventilation whereas 5 patients survived to discharge. Three adverse events [hypoxia (2) and hyperbilirubinemia (1)] were determined to be severe in nature. CONCLUSIONS: These data suggest that PLV may be performed safely with few related severe adverse effects. Improvement in gas exchange was observed in this series of adult patients over the 48 hours after initiation of PLV.

Adult↗
PubMed1998

Comparison of questionnaire-based and serological screening for natural latex allergy in children with neurogenic bladder dysfunction.

OBJECTIVE: We evaluated the prevalence of sensitization to natural latex in children with congenital neurogenic bladder dysfunction by patient history and serological examinations. METHOD: 50 consecutive children aged from 6 months to 17 years were included into the study which comprised questionnaires of patient and family histories, serological screening tests for common inhalational allergens (CAP SX1), and determination of total serum immunoglobulin E (IgE) and of latex-specific serum IgE by solid-phase immunoassays. RESULTS: 5 children had a history of allergic reactions to natural latex; a history of atopic disposition or allergic reactions other than to latex was present in 16 children. In 10 children, latex-specific IgE levels were elevated: > 0.35 kU/l; 6 out of these 10 children had no history of allergic reactions to latex. Elevated latex-specific IgE values were statistically correlated with the number of past operations (p < 0.006) and a history of atopic or allergic reactions (p < 0.0067). Clean intermittent catheterization, however, was not associated with an increased risk of latex allergy (p = 0.408). CONCLUSION: When screening children with congenital neurogenic bladder dysfunction for sensitization to natural latex, serological examinations of latex-specific IgE in addition to patient history may better identify patients at risk.

Adolescent↗
PubMed1997

[Long-term results of Stamey bladder neck suspension. Effect of data analysis technique on surgical result].

The methodology used to assess postoperative continence has a significant effect on the outcome of incontinence surgery. Retrospective chart review studies consistently report higher success rates than patient questionnaire-based outcome analyses. The purpose of this study was to evaluate the true long-term results of the Stamey bladder neck suspension procedure using an anonymous patient questionnaire. Of 172 consecutive patients, 138 (80.2%) returned a questionnaire on long-term continence, complications and subjective satisfaction with the operative result. With a mean follow-up of 66 months, 65 of 130 evaluable patients (50.0%) remained completely continent while 15 (11.5%) never became continent and 50 (38.5%) had recurrent incontinence 6-90 months postoperatively. Almost two-thirds of the patients felt either cured or substantially improved more than 5 years after surgery. Stamey bladder neck suspension leads to a persistently improved quality of life despite a considerable failure rate. Our study demonstrates the advantages of patient questionnaires for outcome analyses and emphasizes the need for standardisation and validation to allow comparisons between the results of different incontinence procedure in the future.

Adult↗
PubMed1997

Primary hyperoxaluria type 2.

UNLABELLED: Primary hyperoxaluria type 2 (PH2) is a rare disease with only 24 patients reported in the literature so far. It should be considered in any patient presenting with urolithiasis or nephrocalcinosis due to hyperoxaluria. The metabolic defect is deficiency of D-glycerate dehydrogenase/glyoxylate reductase leading to characteristic hyperoxaluria and excretion of L-glycerate, the cornerstone of diagnosis of PH 2. Although development of terminal renal failure seems to be less prevalent than in PH 1, recent reports indicate that chronic as well as terminal renal insufficiency may occur. Therefore specific therapeutic measures should aim at reduction of urinary calcium oxalate saturation by potassium citrate or pyrophosphate to reduce the incidence of nephrolithiasis and nephrocalcinosis and thus improve renal survival. Secondary complications (obstruction, urinary tract infections and pyelonephritis) must be avoided. In patients with terminal renal failure isolated renal transplantation seems to carry a high risk of disease recurrence. CONCLUSION: PH 2 is a rare but important cause of urolithiasis and nephrocalcinosis; long-term follow up is necessary, since the renal prognosis may be worse than previously anticipated.

Humans↗