Orthotopic bladder substitution by detubularized sigmoid using a new method of neovesico-urethral anastomosis.
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Turner syndrome (TS) is a common disorder (1/2500 and 1/5000 female births) which is diagnosed at birth in approximately 20% of patients and during childhood (usually due to growth retardation) or later, (due to lack of pubertal development) for the remaining patients. Here we present a cytogenetic and molecular analysis of three monozygotic sisters. The diagnosis of TS was done for one of them (patient 1) who presented with a typical Turner phenotype. A first karyotype was established as normal and a second karyotype (carried out on 200 cells) revealed a 45,X/46,XX mosaicism with 6% of cells with a 45,X karyotype. Lymphocyte karyotype analysis showed the same mosaicism pattern for the two other sisters, one of them exhibiting only a mild (patient 2) and the other no clinical features of Turner syndrome (patient 3). Karyotype analysis was this time conducted on fibroblasts and showed that the 45,X/46,XX mosaicism pattern correlated with the clinical phenotype with 99, 43 and 3% of 45,X cells in patients 1, 2, and 3, respectively. These data suggest that different tissues other than lymphocytes should be subjected to a karyotype analysis when the observed genotype does not correlate with the clinical phenotype.
The model of keratinocytes cultured on a synthetic porous membrane at the air-liquid interface leads to the formation of a pluristratified and cornified epidermis with histological and biochemical characteristics near those observed in vivo. In the present study, we evaluated the effect of proliferative endothelial cells on epidermalization. Keratinocytes were grown in three culture conditions: in defined medium (DM; control), in medium previously conditioned by proliferative endothelial cells (CM) and in medium with proliferative endothelial cells (pEC). The structures of reconstructed epidermis were analyzed by electron microscopy, their biochemistry by DNA, protein and cytokine analyses and finally their functionality was evaluated by estradiol and water absorption testing. Ultrastructural analysis showed a well-developed and cornified epidermis for each culture condition. In addition, living epidermis was thinner in the presence of endothelial cells, revealing faster epidermal differentiation. DNA and protein analyses were in accordance with these results. Secreted soluble factors varied according to culture conditions. At 37 degreesC, the permeability of reconstructed epidermis in DM, in CM or with pEC was 5- to 10-fold higher than that of native human epidermis with both tracers. Laminin coating of the inserts led to similar absorption results except for the DM where the barrier function to estradiol was decreased 2-fold. At 32 degreesC, reconstructed and native epidermis were, respectively, 1.5- and 2-fold less permeable to estradiol compared to 37 degreesC. In conclusion, this model is adequate for fundamental and pharmacological studies since it allows the study of interactions between two cell types without their direct contact as well as percutaneous absorption tests directly performed in the modified culture chamber.
OBJECTIVE: The aim of this study is to compare vesical and renal calcium oxalate crystalluria in an attempt to correlate crystal formation with chemical composition and calcium oxalate saturation of renal urine. MATERIAL AND METHODS: Urine specimens were directly collected from the bladder and the kidney, of 11 stone formers and 11 control subjects under general anesthesia. The type of crystals present in urine as well as their size, number by cubic millimeter and state of aggregation were determined. In addition, calcium, magnesium, sodium, chloride, phosphate, citrate, oxalate, pyrophosphate and uric acid were measured in order to evaluate the calcium saturation status (EQUIL V program). RESULTS: Calcium oxalate crystals were detected in 3 stone formers (27%) and 2 control subjects (18%) in vesical urine and in 4 stone formers (36%) and 3 control subjects (27%) in renal urine. Only 2 stone formers presented with simultaneous renal and vesical crystalluria. Subjects of the two groups with and without renal crystalluria were compared in terms of chemical composition and calcium oxalate saturation of renal urine. Crystalluric subjects (n = 7) had significantly higher uricosuria (p = 0.02), calciuria (p = 0.04), magnesiuria (p = 0.04) and calcium oxalate molar product (p = 0.05) than noncrystalluric (n = 15); calcium oxalate saturation was similar (p = 0.5). CONCLUSIONS: Beyond theorical considerations on lithogenesis, our observations and in particular the apparent discrepancy between renal and vesical crystalluria pose the problem of the clinical interest of the evaluation of calcium oxalate crystalluria based on freshly voided urine in the assessing the lithogenic risk or in the follow-up of patients who are particularly prone to stone recurrence.
Cystine urinary stones is a relatively rare hereditary disorder of dibasic amino acid transport characterized by frequent recurrences. The management of these stones remains problematical despite the remarkable progress in the urological treatment of upper urinary tract stones. Cystine stones are particularly refractory to extracorporeal shock waves and relatively inaccessible to dye pulsed laser (504 nm). Apart from this exception, endourological techniques often represent the most appropriate therapeutic solution, but they are associated with significant morbidity. The physicochemical characteristics of these stones also allow dissolution by urinary alkalinization or the formation of disulfide compounds. In parallel with oral treatments, which constitute the basis of prevention of recurrence, dissolution can be obtained by direct perfusion of the urinary tract. This approach often requires irrigation for several weeks with a risk of the specific complications of catheterization, especially percutaneous catheterization. Prophylaxis, essentially consisting of dilution and dissolution of urinary cystine, raises the problem of the potential adverse effects of drug treatment. Cystinuria is easily detectable and can be investigated either systematically or only in the families concerned. However, the incidence as well as the frequently benign nature of cystinuria tend to limit its value and its indications.
Aneurysmal dilatation of a patent ductus arteriosus is a rare condition. One hundred and fifty three cases have been reported, of which 59 (39%) were operated, with an operative mortality of 16%. The authors report a case detected in a premature neonate on chest X-ray showing a mass in the mid-mediastinal region. Surgical resection was undertaken by video-thoracoscopy. Peroperative analysis of the resected specimen suggested a vascular structure and this was confirmed by histological study, which showed spontaneous aneurysm of a thrombosed patent ductus arteriosus or cystic residue of a patent ductus arteriousus. The authors underline the importance of peroperative puncture of structures of uncertain origin and the possibility of conversion of such a procedure to that of an extreme emergency.
OBJECTIVE: To evaluate the prognosis of stage pT3bM0 invasive urothelial bladder tumours treated by cystectomy alone or combined with adjuvant chemotherapy according to the MVAC protocol (methotrexate, vinblastine, adriamycin and cisplatin). MATERIAL AND METHODS: From 1987 to 1996, 90 patients with stage pT3M0 urothelial bladder tumours were treated with isolated cystectomy (n = 69) or followed by MVAC chemotherapy (n = 21). Lymph node stage was N0 (n = 55), N+ (n = 29) or Nx (n = 6). Essentially selected because of their good general status, patients treated with chemotherapy had a lymph node stage N0 (n = 7) or N+ (n = 14). Chemotherapy had to be suspended in 2 cases and with a fatal outcome during treatment in 4 cases, due to tumour progression, surgical complication or bone marrow aplasia. RESULTS: 65 deaths have occurred with a follow-up of 2 to 120 months (m = 15), including 2 postoperative deaths, 39 cancer deaths and 14 intercurrent deaths. The 1-year, 2-year and 5-year actuarial survival rates were 70%, 48% and 19% for stage N0 and 54%, 25% and 3% for stage N+, respectively, with corresponding median survivals of 20 and 12 months (p < 0.005). The recurrence rate increased from 40% at stage N0 to 62% at stage N+ (p = 0.05), and the corresponding recurrence-free survivals were 16 months and 7 months (p < 0.02). The median survival without chemotherapy ranged from 11 months at stage N+ to 20 months at stage N0 and, with chemotherapy, from 19 months at stage N+ to 67 months at stage N0. The median recurrence-free survival with and without chemotherapy, was 43 months and 17 months at stage N0 and 12 months and 7 months at stage N+. CONCLUSION: The prognosis after cystectomy for stage pT3b bladder cancer is severe, especially in the presence of lymph node involvement. Adjuvant chemotherapy according to the MVAC protocol tends to improve survival, especially recurrence-free survival, and appears beneficial at stage N0. However, the value of this adjuvant treatment, which is associated with a high specific morbidity appears to be more relative at stage N+.
The authors report the first case of bladder paraganglioma in a child admitted to their department successfully treated by partial cystectomy. The diagnosis was established histologically, after failure of clinical diagnosis. Retrospectively' the authors think that preoperative diagnosis should have been possible and emphasize the importance of a thorough clinical examination.
In the present report we studied the interaction between the skeletal muscle ryanodine receptor and the ubiquitous S100A1 Ca2+ binding protein. S100A1 did not affect equilibrium [3H]ryanodine binding to purified rabbit skeletal muscle terminal cisternae at 100 microM free [Ca2+]. At nanomolar free [Ca2+], however, S100A1 activated by 40 +/- 6.7% (mean +/- SE, n = 5) the [3H]ryanodine binding activity; the half-maximal concentration for stimulation of [3H]ryanodine binding was approximately 70 nM, a value well below the estimated S100A1 concentration in skeletal muscle fibers. Scatchard analysis of [3H]ryanodine binding performed in the presence of 100 microM EGTA indicates that S100A1 increases the apparent affinity of the receptor for ryanodine (Kd = 191 vs 383 nM in the presence and in the absence of 100 nM S100A1, respectively). The effect of S100A1 was also tested on the single-channel gating properties of the purified ryanodine receptor after reconstitution into a lipid planar bilayer. Currents carried by purified ryanodine receptor channels were modulated by both cis Ca2+ and ruthenium red. In the presence of nanomolar [Ca2+], S100A1 activated the channel by increasing (6.0 +/- 2.8)-fold (mean +/- SE, n = 3) the normalized open probability. The interaction between S100A1 and the purified RYR was verified using the optical biosensor BIAcore: we show that the two proteins interact directly both at millimolar and at nanomolar calcium concentrations. We next mapped the regions of the skeletal muscle RYR involved in the interaction with S100A1 by performing ligand overlays on a panel RYR of fusion proteins in the presence of 100 nM S100A1. Our results indicate that the skeletal muscle RYR contains three potential S100A1 binding domains. Binding of S100A1 to the RYR fusion proteins occurred at both nanomolar and millimolar free [Ca2+]. S100A1 binding domain 1 binds the ligand in the presence of 1 mM free [Ca2+] or 1 mM EGTA. Maximal binding to S100A1#2 was achieved in the presence of 1 mM free [Ca2+]. The S100A1#3 domain, which overlaps with calcium-dependent calmodulin binding domain 3 (CaM 3), exhibits weak and strong S100A1 binding activity in the presence of either millimolar or nanomolar Ca2+, respectively. The interaction between S100A1 and the purified RYR complex was also investigated by affinity chromatography: in the presence of nanomolar Ca2+, we observed binding of native RYR complex to S100A1-conjugated Sepharose. This interaction could be inhibited by the presence of RYR polypeptides encompassing S100A1 binding sites S100A1#1, S100A1#2, and S100A1#3.
The protease prostate-specific antigen (PSA) is a marker widely used clinically for monitoring prostatic malignancies. Under normal conditions, this enzyme is mainly involved in the post ejaculation degradation of the major human seminal protein, the seminal plasma motility inhibitor precursor/semenogelin I (SPMIP/SgI), which is the predominant protein component of human semen coagulum. PSA primary structure and activity on synthetic substrates predict a chymotrypsin-like activity whose specificity remains to be established. The present study was aimed at characterizing the proteolytic processing of the SPMIP/SgI by PSA. Purified SPMIP/SgI was incubated with PSA in the presence or absence of protease inhibitors. General serine protease inhibitors, heavy metal cations (Zn2+ and Hg2+), and the heavy metal chelator 1,10-phenanthroline partially or totally inhibited the proteolytic activity of PSA toward SPMIP/SgI. Under identical conditions, other proteins, such as bovine serum albumin, ovalbumin, and casein, were very poor substrates for PSA. Hydrolysis products were separated by reverse-phase high-performance liquid chromatography, assayed for sperm motility inhibitory activity, and analyzed by immunoblotting and mass spectrometry. The region responsible for the sperm motility inhibitory activity and containing an SPMI antiserum epitope was localized to the N-terminal portion of the molecule between residues 85 and 136. On the other hand, a monoclonal antibody against a seminal vesicle-specific antigen (MHS-5) recognized fragments derived from the central part of the SPMIP/SgI (residues 198-223). PSA hydrolysis occurred almost exclusively at either leucine or tyrosine residues, demonstrating directly for the first time a restricted chymotrypsin-like activity on a physiological substrate. The results suggest that PSA is the main enzyme responsible for the processing of SPMIP/SgI in human semen and that this protease manifests unusual specificity with respect to hydrolyzable substrates and sites of hydrolysis.
OBJECTIVE: To compare random urine protein-creatinine ratios with 24-hour urine protein excretion rates in patients hospitalized with hypertensive disorders in pregnancy. METHODS: All hospitalized, hypertensive patients requiring 24-hour urine protein excretion collections were eligible for the study. During the 24-hour urine collection a separate 2-mL aliquot was taken for a protein and creatinine determination. RESULTS: Seventy-one samples were collected from patients with the following diagnoses: gestational hypertension (n = 56), preexisting hypertension and superimposed gestational hypertension (n = 11), and syndrome of hemolysis, elevated liver enzymes and low platelets (n = 4). The correlation coefficient between the random protein-creatinine ratio and the 24-hour urine protein excretion was 0.94. Calculated excretion rates with at least 300 mg protein in 24 hours had a sensitivity of 0.93, specificity of 0.90, and positive and negative predictive values of 0.87 and 0.95, respectively. For those samples with calculated excretion rates at least 5 g protein in 24 hours, the sensitivity was 1.00, specificity was 0.99, and positive and negative predictive values were 0.75 and 0.99, respectively. CONCLUSION: In nonambulatory hypertensive pregnant patients, there is a strong correlation between random voided protein-creatinine ratios and 24-hour urine protein excretions.
AIMS: Interleukin 6 (IL-6) is expressed in the majority of renal cell carcinomas and has an important role in the proliferation of some renal cell carcinoma cell lines. This action is mediated by two membrane proteins, gp80 (the IL-6 receptor; IL-6R), which binds IL-6, and gp130, which transduces the signal. The soluble form of gp80 (sIL-6R) is able to activate gp130 when complexed to the IL-6 molecule. These considerations prompted an investigation of IL-6R expression in this malignancy. IL-6, C reactive protein (CRP), and sIL-6R were also measured in serum and correlated to clinical and pathological features. METHODS: Immunostaining was performed on cryostat sections from renal cell carcinoma tumours with M91, an anti-IL-6R monoclonal antibody, using the alkaline phosphatase antialkaline phosphatase technique. The proliferation index was measured using the KI-67 monoclonal antibody. CRP, IL-6, and sIL-6R were measured in serum before nephrectomy, using an immunoenzymatic or immunoradiometric assay. RESULTS: There were significant differences in survival in patients with tumours larger than 8 cm, metastasis at diagnosis, high nuclear grade tumours, detectable serum concentrations of IL-6 (correlated to CRP serum concentration), more than 4% proliferating cells, and the presence of the IL-6R in situ. Furthermore, the serum IL-6 concentration correlated with tumour size and stage. The mean serum sIL-6R concentration was not significantly different from that observed in 40 normal subjects. Tumour IL-6R expression was present in 10 samples. There was a significant association between the presence of the IL-6 receptor in tumours and tumour stage, nuclear grade, proliferation index, and serum IL-6. CONCLUSIONS: This study revealed the importance of IL-6/CRP and IL-6R expression in situ as potential new prognostic factors and opens the way to new therapeutic strategies in renal cell carcinoma.
To evaluate the epidermal barrier function of in vitro reconstructed epidermis, we measured the penetration of estradiol and water across human keratinocytes cultured in defined medium (DM), in the presence of proliferative fibroblasts (pF) or conditioned medium derived from pF, at the air-liquid interface on synthetic porous membrane, noncoated or coated with laminin, fibronectin, type I collagen or type IV collagen. Ultrastructural analysis showed a well-developed stratum corneum whatever the culture conditions. The permeability of reconstructed epidermis in DM on a noncoated porous membrane was 5- to 10-fold higher than human native epidermis, with both tracers. No significant change in barrier function was observed whatever the culture conditions.
OBJECTIVE: To evaluate the crystalline composition of encrustations on double-J ureteric stents in order to prevent their formation on the base of urolithiasis prophylaxis. PATIENTS: 40 patients had a polyurethane double-J ureteric stent inserted between June 1994 and March 1995. Group 1 comprised 22 stone formers whose stents were placed in support of endourological treatment or extracorporeal shock wave lithotripsy of renal or ureteric calculi. Group 2 comprised 18 patients whose stents were inserted for advanced and obstructive malignancy (n = 8) or as an adjunct to reconstructive surgery or endourological techniques (n = 10). After removal, stents were examined for encrustation and obstruction. A biochemical semiquantitative analysis was performed for deposits > 5 mg, and smaller sediments were examined with a polarizing optical microscope. RESULTS: The incidence of encrustation was significantly higher (p = 0.009) for stone formers. In addition, in this group indwelling times of encrusted and obstructed stents were significantly shorter (p = 0.03 and 0.02, respectively). No particular relationship was found between the incidence of encrustation and indwelling times for stone formers. Conversely, for patients without urolithiasis, indwelling times were significantly longer for encrusted or obstructed stents than for unaffected ones (p = 0.05 and 0.02, respectively). Biochemical and optical analyses of encrustations mainly revealed calcium oxalate, calcium phosphate and ammonium magnesium phosphate. Calcium oxalate was the main crystalline phase, especially in the absence of urinary infection. CONCLUSION: Calcium oxalate represents the principal component of double-J ureteric stent encrustations. Thus, prophylaxis of encrustation may consist of preventive measures usually applied in cases of recurrent idiopathic calcium oxalate urolithiasis.
OBJECTIVES: The afferent innervation of ileoanal pouches plays an important role in perception and in reflex regulation of pouch function. We aimed to characterize afferent nerve function in ileoanal pouches and thereby explain the clinical presentation of patients with such pouches. METHODS: In eight patients who underwent surgery for active ulcerative colitis, perception of balloon distention of the pouch was measured using an electronic visceral distention device. In addition, pouch compliance and ileoanal reflex function were determined. Studies were performed during the initial stage of pouch creation (pouch without connection to proximal ileum, study 1) and a mean of 6.6 wk (range = 2-12 wk) after the second stage of the surgery (proximal ileum in continuity with the pouch, study 2). Nine normal volunteers made up the control group. RESULTS: When expressed as intrapouch pressure, thresholds for stool and discomfort sensations were similar in patients with a pouch and controls. However, during ramp distention, patients with a pouch had a lower volume threshold for stool sensation (143 +/- 45 ml vs 300 +/- 30 ml, p = 0.009). The initial sensation of discomfort was experienced in the perineum at the S3 dermatome in all eight patients. At higher distention pressures, all patients with a pouch referred sensations to the abdomen, as opposed to only one of nine controls with such a referral pattern (p < 0.0001). Pouch compliance was markedly reduced during study 1 but normalized after continuity was established with the proximal ileum. Anal sphincter function in patients with a pouch was similar to that in controls. CONCLUSIONS: Afferent pathways from both the ileum and rectum play a role in the mediation of sensations during mechanical distention of the ileoanal pouch and can explain a number of clinical features of patients with a pouch. The thresholds for activation of these pathways are not significantly altered by long-standing colorectal inflammation or by creation of the ileoanal pouch. Continuity with the proximal ileum significantly influences pouch compliance.
OBJECTIVE: To assess the value of retrograde endoscopic lithotripsy for very large pyelocaliceal stones. MATERIAL AND METHODS: Eighteen patients between the ages of 28 and 80 years (mean : 52) and presenting a staghorn renal calculus (n = 7) or with a maximal diameter greater than or equal to 20 mm (n = 11) were initially managed by rigid or flexible retrograde ureterorenoscopy, with ballistic (Lithoclast) or electrohydraulic (Riwolith) stone fragmentation. In 16 cases (89%), an additional extracorporeal shock-wave lithotripsy (ESWL) session was performed immediately after the endoscopic procedure. Early complications consisted of 3 cases of bacteraemic discharge rapîdly responding to medical treatment and 1 death from septic shock on the 8th postoperative day. Twelve patients (67%) were subsequently treated by ureteroscopy (n = 4) or ESWL (n = 11). RESULTS: 17 patients were evaluated after this therapeutic procedure, with a follow-up of 3 to 6 months (mean : 4). Stone elimination was complete for 8 patients (47%), 3 of whom initially presented a staghorn calculus. A residual stone was observed in 9 cases (53%), with a maximal diameter < 5 mm in 7 cases (41%). One patient (5%) underwent secondary percutaneous nephrolithotomy. CONCLUSION: Technological progress has clearly facilitated the ureteroscopic approach to very large pyelocaliceal stones, but fragmentation and stone elimination remain problematical. This unconventional approach constitutes a potential field of technical progress, but does not represent a really efficient alternative to PCNL at the present time.
This is a general survey of soil quality degradation and its consequences on human health and environment quality. The first part deals with the large complexity and reactivity of soil and its specific position as an environmental interface. The origin and behaviour of the main pollutant accumulated in soils are treated in the second and third parts. A special attention is paid to the main pollutants i.e., anions, cations (trace elements), pesticides, persistent organic pollutants (POP), bacteria, DNA... As they are closely linked to the soil constituents (clays, humus...), their real toxicity or effectiveness is examined. In the last part, the risks of soil pollutants for the environment and human health are shortly considered. Direct risks for health are few and mainly concern the ingestion of polluted soil by children; as regards the ecotoxicological and toxicological risks, the Critical pollutant loads have to be defined. The main risks are indirect and involve pollution of either the food chain or of the water and sediments by vertical or lateral (erosion) transfers.
OBJECTIVE: To evaluate the efficacy of the EDAP LT 02 lithotriptor for the treatment of lower pole nephrolithiasis. METHODS: From January 1994 to September 1995, 91 patients presenting with solitary radiopaque calculi of the lower pole calix were treated by piezoelectric extracorporeal shock-wave lithotripsy (ESWL) with the EDAP LT 02. Among them, 82 were available for follow-up. The stones' largest diameter of these patients varied from 5 to 15 mm (mean = 8.1). Indications for ESWL were pain in 63 (77%), hematuria in 5 (6%), associated infection in 6 (7.5%) and stone size in 8 (10%) asymptomatic patients. Stone localization was assessed as very easy in 74 cases (90%) and difficult in 8 cases (10%) but no intraoperative IVP was needed. ESWL sessions were performed with intravenous sedo-analgesia in 69 cases (80%) and general anesthesia in 17 cases (20%). After ESWL we advised patients to combine a diuresis with postural drainage. RESULTS: Most patients were treated with one session of ESWL: none required more that two (mean = 1.05). The mean hospital stay for one session was 1.2 +/- 0.7 days. Obstructive complication rate was 11% and auxiliary treatments were necessary in 6%. The stone-free rate of in situ piezoelectric ESWL monotherapy was overall 84%, and 95% of patients with pain were cured. CONCLUSION: In the absence of abnormality of the upper urinary tract, the vast majority of small lower pole caliceal stones can be completely removed by piezoelectric ESWL without recourse to more invasive methods.