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Biomedical subjects

A Khoury

Publications and source records attributed to A Khoury.

At least 19 recordsLinked to original sources
PubMed2006

Osteoid osteoma: CT-guided radiofrequency ablation using a water-cooled probe.

BACKGROUND: The purpose of this study was to assess the safety and efficacy of computed tomography (CT) guided percutaneous radiofrequency (RF) ablation of osteoid osteoma by using the water-cooled probe. METHODS: During the period from July 2002 to February 2006, fifty-one patients with osteoid osteomas localized in femur (29), tibia (10), calcaneus (2), talus (2), metatarsus (2), humerus (1), sacrum (1), scapula (1), olecranon (1), patella (1) and thoracic vertebra (1) were treated with CT-guided RF ablation using the Cooltiptrade mark Tyco Healthcare probe. Mean age was 20 (range, 3.5-57 years) and male to female ratio was 36/15. Mean follow-up period was reported 2 years (range, 9-51 months). The procedures were carried out under general anesthesia and the patients were discharged from the hospital within 24 h. RESULTS: Technical failure was reported in only one procedure. Pain disappeared postoperatively in all the patients within 2-3 days and no patients needed analgesic treatment after a week. All patients were allowed fully weight bear and function without limitation after the procedure. Recurrence of the pain was observed in one patient who was treated successfully with a second ablation. Our primary and secondary clinical success rates were 98 and 100% respectively. In one case, wound infection was observed after the procedure as the only post-operative complication in our series. CONCLUSION: CT-guided percutaneous RF ablation of osteoid osteomas using the water-cooled probe is a safe, effective and minimally invasive procedure with high success rate and lack of relapses.

Adolescent↗
PubMed2003

Size and geometry of hepatic radiofrequency lesions.

AIM: To report and compare the size and geometry of hepatic radiofrequency (RF) lesions using the currently available commercial devices. METHODS: A literature search was carried out for the period from January 1st 1990 to June 15th 2003. The commercial suppliers were asked to provide all available data. For each electrode and protocol, size and geometry of single-cycle thermal lesions were registered. RESULTS: No information at all on size and geometry of the inducible lesions was available for 17 of the 28 current commercial electrodes. Many descriptions of RF lesions are limited to the mean transverse diameter. With normal blood flow, diameter of lesions is often smaller than suggested by the length of the electrode tip or the diameter of the deployed prongs. Lesions are rarely perfect spheres but either ellipses or flattened spheres. Distortion of the RF lesion by nearby blood vessels is very common. Fusion of thermal zones between prongs of expandable electrodes can be incomplete. Blood flow interruption using a Pringle maneuver yields larger lesions that are less distorted and more complete. CONCLUSIONS: There is insufficient experimental data for many electrodes that are currently used in patients. RF companies should provide these data before releasing electrodes for use. For those electrodes for which data exist, coagulation lesions are often smaller, less spherical, less complete and less regular than generally presumed. Accurate knowledge of size and geometry of RF lesions is crucial to prevent local recurrence.

Animals↗
PubMed2003

Retroperitoneal laparoscopic vs open partial nephroureterectomy in children.

OBJECTIVES: To compare the results of retroperitoneal laparoscopic with open partial nephroureterectomy. PATIENTS AND METHODS: Laparoscopic retroperitoneal partial nephroureterectomy was undertaken in 15 children (13 upper and two lower poles; median age at the time of surgery 61 months, range 5-212). A three-trocar retroperitoneal approach was used. The polar vessels were identified and either coagulated or clipped before transecting the parenchyma using a harmonic scalpel. An additional 13 consecutive children underwent similar procedures (11 upper and two lower poles) by conventional open surgery (median age at surgery 16 months, range 1.5-72). RESULTS: One patient in the laparoscopy group required conversion to open surgery because of a peritoneal tear and was excluded from the analysis. The mean (range) operative duration was 146 (50-180) and 152 (75-240) min for the open surgery and laparoscopy groups, respectively. The blood loss was minimal (< 20 mL) in both groups. The mean (range) hospital stay was 1.4 (1-3) and 3.9 (3-5) days for the laparoscopy and open groups, respectively (P < 0.001). Eight patients were discharged on the day after the laparoscopic procedure. In addition, of those patients in the laparoscopy group who had a lower-pole partial nephrectomy, one had a urinoma after surgery. CONCLUSION: Laparoscopic retroperitoneal partial nephrectomy is a safe and feasible procedure in children. It requires a similar operative duration to that of an open procedure. The main advantage to the laparoscopic approach is that it significantly decreases the hospital stay compared with that after an open procedure.

Adolescent↗
PubMed2003

Laparoscopic dismembered pyeloplasty by a retroperitoneal approach in children.

OBJECTIVES: To report our experience with dismembered laparoscopic pyeloplasty by a retroperitoneal approach in children with pelvi-ureteric junction (PUJ) obstruction. PATIENTS AND METHODS: Between 1999 and 2002, retroperitoneal laparoscopic dismembered pyeloplasty was attempted in 21 children (one bilateral; mean age 8 years, range 1.7-17). In a flank position with four ports (one of 5 or 10 mm and three of 3 mm), the PUJ was resected and the anastomosis made using 6/0 absorbable sutures. Any redundant renal pelvis was reduced when needed. A JJ stent was inserted in all patients. RESULTS: The procedure could not be completed by laparoscopy in four patients, the main reason being difficulty in completing the anastomosis; in the other 18 patients the procedure was successful. An aberrant crossing vessel was found in nine patients and dismembered pyeloplasty enabled ureteric transposition in all, with no conversion. The mean (range) operative duration was 228 (170-300) min and the mean hospital stay 2.5 (2-4) days. In three patients the JJ stents were not in the bladder at the time of removal by cystoscopy, and ureteroscopy was used to retrieve them. All children returned to full activities within 7 days of surgery. The mean (range) follow-up was 12.7 (2-36) months, with six children followed for> 2 years; all were asymptomatic, with imaging confirming improved hydronephrosis. CONCLUSIONS: These mid-term results confirm that retroperitoneal laparoscopic dismembered pyeloplasty is a safe and feasible approach in children. Although the technique is very demanding it has the advantage of duplicating the principles of the open approach. The long operative duration and high conversion rate might be reduced with experience. Before expanding this approach to younger children, refinements in the anastomotic technique are needed.

Adolescent↗
PubMed2003

Mentored retroperitoneal laparoscopic renal surgery in children: a safe approach to learning.

OBJECTIVE: To review the feasibility of introducing advanced retroperitoneal renal laparoscopic surgery (RRLS) to a paediatric urology division, using the mentorship-training model. Although the scope of practice in paediatric urology is currently adapting endoscopic surgery into daily practice, most paediatric urologists in North America have had no formal training in laparoscopic surgery. METHODS: The study included four paediatric urologists with 3-25 years of practice; none had had any formal laparoscopic training or ever undertaken advanced RRLS. An experienced laparoscopic surgeon (the mentor) assisted the learning surgeons over a year. The initial phases of learning incorporated detailed lectures, visualization through videotapes and 'hands-on' demonstration by the expert in the technique of the standardized steps for each type of surgery. Over 10 months, ablative and reconstructive RRLS was undertaken jointly by the surgeons and the mentor. After this training the surgeons operated independently. To prevent lengthy operations, conversion to open surgery was planned if there was no significant progression after 2 h of laparoscopic surgery. RESULTS: Over the 10 months of mentorship, 36 RRLS procedures were undertaken in 31 patients (28 ablative and eight reconstructive). In all cases the mentored surgeons accomplished both retroperitoneal access and the creation of a working space within the cavity. The group was able to initiate ablative RRLS but the mentor undertook all the reconstructive procedures. After the mentorship period, over 10 months, 12 ablative procedures were undertaken independently, and five other attempts at RRLS failed. CONCLUSION: Although the mentored approach can successfully and safely initiate advanced RRLS in a paediatric urology division, assessing the laparoscopic practice pattern after mentorship in the same group of trainees is warranted. Ablative RRLS is easier to learn for the experienced surgeon, but reconstructive procedures, e.g. pyeloplasty, require a high degree of skill in laparoscopic technique, which may only be acquired through formal training focusing primarily on suturing techniques.

Child↗
PubMed2003

Management of oesophageal perforation after delayed diagnosis: the merit of tissue flap reinforcement.

Oesophageal perforation remains a life-threatening situation and its management represents a challenge for the surgeon, especially if diagnosis has been delayed. In most cases, a surgical approach is indicated. Simple primary repair often result in leakage. In order to avoid leakage, the primary repair should be buttressed with some kind of tissue flap. We here report our experience with 15 esophageal perforations and 10 tissue flap reinforcements performed during the last decade. The different types of tissue flap are illustrated and their results are compared with more aggressive alternatives such as oesophagectomy, T-tube drainage and oesophageal exclusion.

Adolescent↗
PubMed2002

A single-centre long-term outcome analysis of artificial urinary sphincter placement in children.

OBJECTIVE: To retrospectively evaluate the outcome of artificial urinary sphincter (AUS) placement in 79 children, with a mean follow-up of 12.5 years. PATIENTS AND METHODS: A total of 89 children had an AUS placed between 1977 and 1994; complete data were obtained for 79 (63 boys and 16 girls). The indication for AUS placement was urinary incontinence caused by sphincteric deficiency with a stable bladder. Before and after surgery, all patients underwent ultrasonography, voiding cysto-urethrography and urodynamics. Logistic regression was used to assess the factors influencing the outcome. RESULTS: The mean (range) age at surgery was 11.7 (3-18) years. The cause of incontinence was a neuropathic bladder in 74 patients and bladder exstrophy in five. At a mean (range) follow-up of 12.5 (5-22) years, 63 of 79 patients (80%) had an intact AUS. The AUS was removed in 16 patients (20%) because of erosion at a mean (range) of 5.6 (1-11) years after insertion; there were 0.035 revisions per patient-year. Of 63 patients with an AUS in place, 57 (90%) are completely dry and 36 (57%) are using clean intermittent catheterization. Thirteen patients (20%) developed bladder instability during the follow-up, which was managed by anticholinergics in eight, spinal cord de-tethering in three and enterocystoplasty in two. Hydronephrosis occurred in 12 of 126 renal units (10%) that improved after enterocystoplasty or anticholinergics. The overall 10-year survival of the AUS was 79% (78% for early models and 80% for the AMS800); the survival was not affected by age, sex, model, previous bladder neck surgery, augmentation cystoplasty or intermittent catheterization. Bladder exstrophy was associated with lower AUS survival, as four of the five patients affected had the AUS removed secondary to erosion (P = 0.014). CONCLUSION: In children the AUS has a 79% 10-year survival and gives a 90% continence rate. Bladder instability after AUS placement can be managed with anticholinergics or enterocystoplasty. The AUS is not a justifiable treatment option for patients with bladder exstrophy.

Adolescent↗
PubMed2001

A missense mutation in a highly conserved region of CASQ2 is associated with autosomal recessive catecholamine-induced polymorphic ventricular tachycardia in Bedouin families from Israel.

Catecholamine-induced polymorphic ventricular tachycardia (PVT) is characterized by episodes of syncope, seizures, or sudden death, in response to physical activity or emotional stress. Two modes of inheritance have been described: autosomal dominant and autosomal recessive. Mutations in the ryanodine receptor 2 gene (RYR2), which encodes a cardiac sarcoplasmic reticulum (SR) Ca(2+)-release channel, were recently shown to cause the autosomal dominant form of the disease. In the present report, we describe a missense mutation in a highly conserved region of the calsequestrin 2 gene (CASQ2) as the potential cause of the autosomal recessive form. The CASQ2 protein serves as the major Ca(2+) reservoir within the SR of cardiac myocytes and is part of a protein complex that contains the ryanodine receptor. The mutation, which is in full segregation in seven Bedouin families affected by the disorder, converts a negatively charged aspartic acid into a positively charged histidine, in a highly negatively charged domain, and is likely to exert its deleterious effect by disrupting Ca(2+) binding.

Age of Onset↗
PubMed2001

Autosomal recessive catecholamine- or exercise-induced polymorphic ventricular tachycardia: clinical features and assignment of the disease gene to chromosome 1p13-21.

BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (PVT) is characterized by episodes of syncope, seizures, or sudden death in response to physiological or emotional stress. In 2 families with autosomal dominant inheritance, the disease gene was mapped to chromosome 1q42-43. The objectives of this study were to characterize the clinical features of the disease in a Bedouin tribe from Israel and to map the disease gene. METHODS AND RESULTS: In this Bedouin tribe, 9 children (age, 7+/-4 years) from 7 related families have died suddenly during the past decade, and 12 other children suffered from recurrent syncope and seizures starting at the age of 6+/-3 years. Parents of affected individuals were asymptomatic and were all related (first-, second-, or third-degree cousins). Segregation analysis suggested autosomal recessive inheritance. All 12 symptomatic patients and 1 asymptomatic sibling (mean age, 13+/-7 years) were found to have a relative resting bradycardia (64+/-13 bpm, versus 93+/-12 bpm in the unaffected siblings), as well as PVT induced by treadmill or isoproterenol infusion and appearing at a mean sinus rate of 110+/-10 bpm. Patients responded favorably to treatment with beta-blockers. A genome-wide search using polymorphic DNA markers mapped the disease locus to a 16-megabase interval on chromosome 1p13-21. A maximal lod score of 8.24 was obtained with D1S189 at theta=0.00. Sequencing of KCND3, a gene that encodes an I(tO) potassium channel transporter, did not reveal any significant sequence alterations. CONCLUSIONS: This unique form of autosomal recessive PVT affects young children and may be lethal if left untreated. Linkage analysis maps this disorder to chromosome 1p13-21.

Adolescent↗
PubMed2001

The effect of age, gender, risk level and glycosylated hemoglobin in predicting foot amputation in HMO patients with diabetes.

PURPOSE: To determine if age, gender, risk level and glucosylated hemoglobin could predict foot amputations in HMO patients with diabetes. DATA SOURCES: A retrospective chart review of a non-random sample of 27 patients with amputation and 110 randomly selected patients without amputation during the same time period. Chi-square, t-tests and logistic regression were used to analyze the data. CONCLUSIONS: There was no significant difference between the group with amputation and those without amputation in age or gender. However, there was a significant difference between the two groups in the risk stratification (p = .005) and glycosylated hemoglobin levels (p = .058). IMPLICATIONS FOR PRACTICE: Nurse practitioners can effectively screen for amputation risk factors and closely monitor glycosylated hemoglobin levels of diabetic patients to reduce the incidence of vascular complications and related lower extremity amputation.

Adult↗
PubMed2001

A prospective randomized clinical trial to evaluate methods of postoperative care of hypospadias.

PURPOSE: Hypospadias repair is a common operation performed by pediatric urologists. Perhaps the greatest variable and source of controversy of postoperative care is the surgical dressing. We hypothesized that using no dressing would achieve surgically comparable results to those traditionally achieved by a postoperative dressing and it would also simplify postoperative parent delivered home care. Accordingly we designed a prospective randomized clinical trial to compare surgical outcome and postoperative care after hypospadias repair in boys with no dressing and those who received 1 of the 2 most common types of dressing. MATERIALS AND METHODS: In a 12-month period 120 boys with an average age of 2.2 years underwent primary 1-stage hypospadias repair at a single center with 4 participating surgeons. Repair was performed in 60 boys with proximal and 60 with distal hypospadias on an outpatient basis. Ethics and Internal Review Board approval, and informed consent were obtained. Boys were then prospectively randomized to receive no dressing, an adhesive biomembrane dressing or a compressive wrap dressing. Comprehensive instructions on postoperative care were distributed to all families and a questionnaire was distributed to the parents at the initial followup. Surgical outcome was evaluated and questionnaire responses were analyzed. Fisher's exact test was done to test the significance of differences in surgical outcomes and questionnaire responses. RESULTS: A total of 117 boys completed the prospective randomized trial. Surgical staff withdrew 3 cases from randomized selection to place a dressing for postoperative hemostasis. We obtained 101 questionnaires for response analysis. The type or absence of the dressing did not correlate with the need for repeat procedures, urethrocutaneous fistula, or meatal stenosis or regression. Analysis revealed less narcotic use in the no dressing group and fewer telephone calls to the urology nurse, or on-call resident and/or fellow. These findings were statistically significant. In addition, there were more unscheduled visits to the urology clinic, emergency room or primary physician office by boys with than without a dressing. Furthermore, 29% of the parents were not psychologically prepared to remove the dressing and 12% were so reluctant that the dressing was removed at the urology outpatient clinic. CONCLUSIONS: The surgical outcome and rate of adverse events or complications were not compromised without a postoperative dressing. An absent dressing simplified postoperative ambulatory parent delivered home care. We recommend that dressings should be omitted from routine use after hypospadias repair.

Bandages↗
PubMed2001

Outcome analysis of vesicoamniotic shunting in a comprehensive population.

PURPOSE: Prenatal detection of obstructive uropathy is used widely and vesicoamniotic shunting is the accepted procedure in well-defined cases. We present outcomes of vesicoamniotic shunting in a consecutive series from a coordinated prenatal-postnatal treatment program. MATERIALS AND METHODS: From 1989 to 1998 bladder obstruction was identified in 89 fetuses at a multidisciplinary prenatal high risk clinic. Prenatal intervention was considered in cases of bilateral hydronephrosis associated with evidence of bladder outlet obstruction and oligohydramnios. RESULTS: Indications for prenatal shunting were present in 12 patients (13%), and 9 underwent vesicoamniotic shunt insertion between weeks 20 and 28 of gestation. None of the prenatal procedures was associated with preterm labor, chorioamnionitis or urine chemistry values greater than cutoff threshold on bladder tap. Shunts were extruded from 2 fetuses, which required sequential insertion. After prenatal intervention 1 patient elected pregnancy termination and the others proceeded to term. Two neonates died at birth, and 6 survived. The underlying urological diagnosis was posterior urethral valves in 4 newborns, urethral atresia in 1, and prune belly variant and urethral atresia in 1. Of the patients 3 had relatively normal renal function, 2 had severe renal insufficiency and 1 had mild renal impairment. Five newborns are voiding freely and 1 underwent bladder reconstruction and is on intermittent catheterization. None of the patients had pulmonary problems at last followup. CONCLUSIONS: Although vesicoamniotic shunting is effective in reversing oligohydramnios, its ability to achieve sustainable good renal function in infancy is variable. No specific prenatal parameters were effective in predicting eventual good renal function. Pulmonary function cannot be assured with restoration of amniotic fluid.

Amnion↗
PubMed2001

Efficacy of endoscopic subureteral polydimethylsiloxane injection for treatment of vesicoureteral reflux in children: a North American clinical report.

PURPOSE: Subureteral injection of bulking agents is an accepted surgical treatment of vesicoureteral reflux in children. Polydimethylsiloxane, a silicone elastomer, is an ideal agent because of bulky consistency, lack of migration, minimal local inflammatory reaction and is safe in laboratory animals. We record our experience with endoscopic subureteral polydimethylsiloxane injection in children for vesicoureteral reflux. MATERIALS AND METHODS: During a 2-year period 16 boys and 58 girls, with an average age of 8 years, with 112 refluxing ureters underwent endoscopic subureteral polydimethylsiloxane injection to treat vesicoureteral reflux. Vesicoureteral reflux was grade I in 8, II in 43, III in 50, IV in 10 and V in 1 ureter. Operative indications were breakthrough urinary tract infection in 29 children, nonresolution of reflux 38 and high grade reflux 7. All procedures were on an outpatient basis and performed with patient under general anesthesia. All children had a postoperative ultrasound and voiding cystourethrogram at 12 weeks. Followup was from 6 to 24 months. RESULTS: Overall, reflux was corrected in 90 (81%) ureters and 56 (76%) children after a single injection. With repeat injection reflux was corrected in 101 (90%) ureters and 63 (85%) children. Correction by grade was 85%, 84%, 80%, 45% and 0% for grades I to V, respectively. With repeat injection correction was 100%, 92%, 90% and 55% for grades I to IV, respectively. There were no surgical complications. De novo contralateral reflux developed in 2 (3%) children. There were 3 (4%) children who required open ureteral reimplantation for failed injection. Detection of the polydimethylsiloxane implant by followup ultrasound was 89% sensitive and 86% specific for the correction of reflux. CONCLUSIONS: Endoscopic subureteral polydimethylsiloxane injection is an effective treatment of vesicoureteral reflux in children. The procedure is safe with low associated morbidity. The presence of the polydimethylsiloxane implant can be documented accurately by ultrasound, and there is a strong correlation between implant stability and correction of reflux.

Adolescent↗
PubMed2001

Reliability of the pediatric dysfunctional voiding symptom score in monitoring response to behavioral modification.

PURPOSE: We previously evaluated the performance of a newly devised dysfunctional voiding symptom score to quantify abnormal voiding behaviors in children. The symptom score ranges from 0 to 30 with higher scores indicating greater severity. In this study, we test the performance of the symptom score in monitoring effectiveness and compliance with a program of behavioral modification. MATERIALS AND METHODS: One hundred four patients (age 3-10 years) were clinically diagnosed as having dysfunctional voiding (DV). At initial visit, after their baseline DV symptom scores were tabulated, they were introduced to a program of behavioral modification that included both verbal and written instructions. After an average follow up of 5 months, all families were mailed questionnaires to: a) assess whether the child was compliant with bladder retraining, and b) request the completion of a second DV symptom-scoring sheet. The change in symptom score was correlated with treatment compliance. Statistical analysis of the data was done using non-parametric method (Mann-Whitney U test). RESULTS: Forty-eight out of 104 children (46%) completed the mailed questionnaires. Twenty-eight (Group A1) were compliant with the behavioral modification program, and the remaining 20 children (Group A2) were not compliant. Although the median score at initial evaluation was similar in both groups (15 versus 14.5 respectively), scores in last follow up were significantly lower in Group A1 than the initial scores (6 versus 14.5 respectively). The scores in last follow-up in Group A2 were not significantly different from their initial symptom scores (median 11 versus 14.5 respectively). CONCLUSION: The DV symptom score reliably and quantitatively reflects improvement in voiding symptoms in patients compliant with behavioral modification. Patients non-compliant with a bladder retraining program may be identified by lack of decrease in their DV symptom score. Key Words: voiding dysfunction, pediatric, behavioral modification, symptom score

Behavior Therapy↗
PubMed2000

Outcomes of primary valve ablation versus urinary tract diversion in patients with posterior urethral valves.

OBJECTIVES: Although valve ablation is the treatment of choice for patients with posterior urethral valves, debate continues as to the role of urinary diversion. We sought to retrospectively compare the clinical and radiologic outcomes between valve ablation and urinary diversion for patients with posterior urethral valves. METHODS: We retrospectively reviewed the records of 50 consecutive patients with posterior urethral valves since January 1995. On the basis of the initial renal function and radiologic findings, patients were divided into three groups: group 1, normal renal function and radiologically normal upper tracts; group 2, normal renal function with hydronephrosis and/or reflux; and group 3, azotemia with hydronephrosis or reflux. RESULTS: All 22 patients in group 1 were treated with valve ablation. After a mean follow-up of 32 months, these children had normal renal function and no evidence of upper tract deterioration. All 13 patients in group 2 were also treated with valve ablation. The radiologic abnormalities (hydronephrosis, reflux) resolved in 50% of cases, with an average follow-up of 28 months. Of the 15 patients in group 3, 7 underwent valve ablation and 8 underwent urinary diversion. Urinary diversion was performed in patients with renal deterioration and severe hydronephrosis and/or high-grade reflux. Renal function returned to normal in all patients who underwent valve ablation except one; renal function returned to normal in only 3 of 8 patients who underwent urinary diversion. Radiologically, the severity of the hydronephrosis and reflux was downgraded in patients who underwent valve ablation but not in the diverted group. CONCLUSIONS: Valve ablation is the mainstay of treatment for patients with posterior urethral valves. Prenatal and postnatal factors, such as renal dysplasia and urinary tract infection, respectively, rather than the posterior valve treatment dictate the long-term renal and radiologic outcomes.

Adolescent↗
PubMed2000

Regulation of HMG-CoA reductase degradation requires the P-type ATPase Cod1p/Spf1p.

The integral ER membrane protein HMG-CoA reductase (HMGR) is a key enzyme of the mevalonate pathway from which sterols and other essential molecules are produced. HMGR degradation occurs in the ER and is regulated by mevalonate-derived signals. Little is known about the mechanisms responsible for regulating HMGR degradation. The yeast Hmg2p isozyme of HMGR undergoes regulated degradation in a manner very similar to mammalian HMGR, allowing us to isolate mutants deficient in regulating Hmg2p stability. We call these mutants cod mutants for the control of HMG-CoA reductase degradation. With this screen, we have identified the first gene of this class, COD1, which encodes a P-type ATPase and is identical to SPF1. Our data suggested that Cod1p is a calcium transporter required for regulating Hmg2p degradation. This role for Cod1p is distinctly different from that of the well-characterized Ca(2+) P-type ATPase Pmr1p which is neither required for Hmg2p degradation nor its control. The identification of Cod1p is especially intriguing in light of the role Ca(2+) plays in the regulated degradation of mammalian HMGR.

ATP-Binding Cassette Transporters↗
PubMed2000

The dysfunctional voiding scoring system: quantitative standardization of dysfunctional voiding symptoms in children.

PURPOSE: Academic research on pediatric nonneurogenic voiding dysfunction has long been hampered by the lack of a standardized reporting system for voiding symptoms. We evaluated the performance of a newly devised, objective instrument to quantify or grade the severity of abnormal voiding behaviors of children. MATERIALS AND METHODS: There were 10 voiding dysfunction parameters that were assigned scores of 0 to 3 according to prevalence, and possible total scores ranged from 0 to 30. The Dysfunctional Voiding Symptom Score was completed by 2 groups of patients. Group 1 consisted of patients 3 to 10 years old presenting to the pediatric urology clinic with a history of diurnal urinary incontinence, urinary tract infections or abnormal voiding habits. Group 2 consisted of an age matched cohort with no history of urological complaints presenting to hospital clinics outside of urology. Patients diagnosed with organic or anatomical disease, such as posterior urethral valves or meningomyelocele, were excluded from our analysis. RESULTS: Group 1 consisted of 104 patients (female-to-male ratio 4:1) with a median symptom score of 14 and group 2 consisted of 54 patients (female-to-male ratio 1.3:1) with a median score of 4. The dysfunctional voiding odds ratio was 2.93 for females compared to that of males. Using receiver operating characteristics the optimum cutoff score was 6.026 (sensitivity 92.77% and specificity 87.09%) for females and 9.02 (sensitivity of 80.95% and specificity of 91. 30%) for males. In addition, we found certain questions to be more reflective than others of dysfunctional voiding symptoms in our population. CONCLUSIONS: The Dysfunctional Voiding Symptom Score appears to provide accurate and objective, that is, numerical, grading of voiding behaviors of children. Comparative research studies of dysfunctional voiding diagnosis and response to therapy as well as objective measurements of treatment efficacy and outcomes analysis should be aided greatly by this system.

Child↗