Performance of Patient-Reported Outcomes Measurement Information System (PROMIS) scores compared with legacy metrics in evaluating outcomes after surgical treatment for osteochondritis dissecans of the humeral capitellum.

BACKGROUND: Patient-Reported Outcomes Measurement Information System (PROMIS) scores have not previously been used to measure long-term outcomes in operatively treated capitellar osteochondritis dissecans (OCD) patients. The aims of our study were to (1) assess patients' long-term outcomes using PROMIS scores, (2) compare the performance of PROMIS with other validated elbow legacy metrics, and (3) evaluate ceiling and floor effects in these outcome measures in patients undergoing surgical treatment for capitellar OCD. METHODS: We evaluated demographic characteristics, procedure details, preoperative PROMIS scores, and associated sports information in surgically treated pediatric capitellar OCD patients. An online survey was sent to the study participants, including the Kerlan-Jobe Orthopaedic Clinic (KJOC) shoulder and elbow score, the quick Disabilities of the Arm, Shoulder and Hand questionnaire, and the Liverpool Elbow Score patient-answered questionnaire, as well as the Mobility, Pain Interference, and Upper Extremity questionnaires from the PROMIS pediatrics bank. Correlations were evaluated between outcome measures. Ceiling and floor effects were evaluated for each outcome measure. RESULTS: Completed surveys were obtained for 57 patients (59 elbows). The mean patient age at surgery was 14 years (range, 10-18 years). The mean follow-up time was 6 years (standard deviation, 5 years; range, 1-18 years). The mean PROMIS Mobility score improved from 41.2 preoperatively to 55.2 postoperatively (P < .001). The mean Pain Interference score decreased from 46.9 preoperatively to 38 postoperatively (P < .001). The mean Upper Extremity score improved from 42.7 preoperatively to 53.2 postoperatively (P < .001). Significant correlations were observed between all legacy metrics and postoperative PROMIS scores (|r| > 0.54, P < .001). Ceiling or floor effects were seen in all legacy metrics and PROMIS scores. The KJOC score was least affected by ceiling or floor effects. CONCLUSION: There is a strong correlation between PROMIS scores and legacy measures evaluating outcomes after surgical management of capitellar OCD. However, large ceiling and floor effects were present in all measures, likely owing to the favorable clinical results. The KJOC score was limited the least by ceiling and floor effects.

Characteristics of AA amyloidosis patients in San Francisco.

BACKGROUND: AA amyloidosis due to subcutaneous injection of drugs of abuse has been described in the USA, but all the existing literature is from more than 20 years ago. There is more recent literature from Europe. We have observed a high incidence of AA amyloidosis in the county hospital in San Francisco. DESIGN: Here, we describe 24 patients who had kidney biopsy-proven AA amyloidosis from our hospital from 1998 to 2013. All the patients were thought to have AA amyloidosis from skin popping of illicit drugs after having exhausted the intravenous route. These patients with biopsy-proven AA amyloidosis were analysed further. RESULTS: All patients were found to have hepatitis C infection, hypertension was not common, most had advanced kidney failure, and acidosis was common as was tubulointerstitial involvement on the kidney biopsy. Other organ involvement included hepatomegaly and splenomegaly in a number of patients; direct myocardial involvement was not seen, but pulmonary hypertension, history of deep vein thrombosis and pulmonary embolism were common. The prognosis of these patients was poor. The mortality rate approached 50% 1 year after biopsy, and most of the patient needed dialysis shortly after diagnosis. Cessation of drug use seemed beneficial but rarely achievable. CONCLUSION: AA amyloidosis from skin popping is common in San Francisco. Most patients with renal involvement end up on dialysis, and mortality rates are exceedingly high.

Lupus-like membranous nephropathy: Is it lupus or not?

BACKGROUND: Membranous glomerulonephritis is typically classified as idiopathic or secondary to systemic lupus erythematosus (SLE), hepatitis B, drugs, toxins, other infections, or malignancy. Not infrequently in some patients without a definite diagnosis of SLE, pathologic features of secondary membranous nephropathy are seen e.g., mesangial and/or subendothelial deposits, tubuloreticular inclusions, and full house immunofluorescence. In these patients, there is uncertainty about the etiology, response to therapy, and prognosis of membranous GN. METHODS: We retrospectively reviewed the charts of 98 patients with membranous GN at San Francisco General Hospital and John Stroger Hospital of Cook County over a 10-year period. Data were collected and analyzed using SPSS.18. RESULTS: Thirty-nine (40 %) had idiopathic membranous GN (Group 1), thirty-six (37 %) had lupus membranous GN (Group 2) and twenty-three (23 %) had some pathological features of secondary membranous GN, but no definite etiology of membranous GN (Group 3). At baseline (at time of renal biopsy) and after mean follow-up of 3.5 years, the average serum creatinine (in mg/dL) in Group 1 was (1.6 ± 1.0 versus 1.6 ± 1.7), Group 2 was (1.8 ± 2.5 versus 1.2 ± 0.9) and Group 3 was (1.1 ± 0.4 versus 1.27 ± 0.83), respectively. For the same time points, the average urine protein to creatinine ratio (g/g) in Group 1 was (9.8 ± 7.1 versus 5.7 ± 6.7), Group 2 was (4.2 ± 3.9 versus 1.7 ± 2.2), and Group 3 was (7.4 ± 5.7 versus 3.1 ± 3.8). In addition, during the follow-up period, eleven of 39 (28 %) in Group 1, two of 36 (6 %) in Group 2, and three of 23 (13 %) in Group 3 progressed to end-stage renal disease and were started on dialysis. CONCLUSIONS: It appears that patients with lupus membranous GN have better renal prognosis than patients with idiopathic membranous GN. The renal prognosis for patients with pathological features of lupus membranous but no diagnosis of systemic lupus (lupus-like membranous GN) falls in between. Further studies are needed to determine if Group 3 patients can (a) definitively be classified as true idiopathic membranous GN or lupus membranous GN or (b) they have a separate disease from either M-type phospholipase A2 receptor membranous nephropathy or systemic lupus-induced membranous nephropathy.

Specialist and primary care physicians' views on barriers to adequate preparation of patients for renal replacement therapy: a qualitative study.

BACKGROUND: Early preparation for renal replacement therapy (RRT) is recommended for patients with advanced chronic kidney disease (CKD), yet many patients initiate RRT urgently and/or are inadequately prepared. METHODS: We conducted audio-recorded, qualitative, directed telephone interviews of nephrology health care providers (n = 10, nephrologists, physician assistants, and nurses) and primary care physicians (PCPs, n = 4) to identify modifiable challenges to optimal RRT preparation to inform future interventions. We recruited providers from public safety-net hospital-based and community-based nephrology and primary care practices. We asked providers open-ended questions to assess their perceived challenges and their views on the role of PCPs and nephrologist-PCP collaboration in patients' RRT preparation. Two independent and trained abstractors coded transcribed audio-recorded interviews and identified major themes. RESULTS: Nephrology providers identified several factors contributing to patients' suboptimal RRT preparation, including health system resources (e.g., limited time for preparation, referral process delays, and poorly integrated nephrology and primary care), provider skills (e.g., their difficulty explaining CKD to patients), and patient attitudes and cultural differences (e.g., their poor understanding and acceptance of their CKD and its treatment options, their low perceived urgency for RRT preparation; their negative perceptions about RRT, lack of trust, or language differences). PCPs desired more involvement in preparation to ensure RRT transitions could be as "smooth as possible", including providing patients with emotional support, helping patients weigh RRT options, and affirming nephrologist recommendations. Both nephrology providers and PCPs desired improved collaboration, including better information exchange and delineation of roles during the RRT preparation process. CONCLUSIONS: Nephrology and primary care providers identified health system resources, provider skills, and patient attitudes and cultural differences as challenges to patients' optimal RRT preparation. Interventions to improve these factors may improve patients' preparation and initiation of optimal RRTs.

Risk of vascular access complications with frequent hemodialysis.

Frequent hemodialysis requires using the vascular access more often than with conventional hemodialysis, but whether this increases the risk for access-related complications is unknown. In two separate trials, we randomly assigned 245 patients to receive in-center daily hemodialysis (6 days per week) or conventional hemodialysis (3 days per week) and 87 patients to receive home nocturnal hemodialysis (6 nights per week) or conventional hemodialysis, for 12 months. The primary vascular access outcome was time to first access event (repair, loss, or access-related hospitalization). Secondary outcomes were time to all repairs and time to all losses. In the Daily Trial, 77 (31%) of 245 patients had a primary outcome event: 33 repairs and 15 losses in the daily group and 17 repairs, 11 losses, and 1 hospitalization in the conventional group. Overall, the risk for a first access event was 76% higher with daily hemodialysis than with conventional hemodialysis (hazard ratio [HR], 1.76; 95% confidence interval [CI], 1.11-2.79; P=0.017); among the 198 patients with an arteriovenous (AV) access at randomization, the risk was 90% higher with daily hemodialysis (HR, 1.90; 95% CI, 1.11-3.25; P=0.02). Daily hemodialysis patients had significantly more total AV access repairs than conventional hemodialysis patients (P=0.011), with 55% of all repairs involving thrombectomy or surgical revision. Losses of AV access did not differ between groups (P=0.58). We observed similar trends in the Nocturnal Trial, although the results were not statistically significant. In conclusion, frequent hemodialysis increases the risk of vascular access complications. The nature of the AV access repairs suggests that this risk likely results from increased hemodialysis frequency rather than heightened surveillance.

In-center hemodialysis six times per week versus three times per week.

BACKGROUND: In this randomized clinical trial, we aimed to determine whether increasing the frequency of in-center hemodialysis would result in beneficial changes in left ventricular mass, self-reported physical health, and other intermediate outcomes among patients undergoing maintenance hemodialysis. METHODS: Patients were randomly assigned to undergo hemodialysis six times per week (frequent hemodialysis, 125 patients) or three times per week (conventional hemodialysis, 120 patients) for 12 months. The two coprimary composite outcomes were death or change (from baseline to 12 months) in left ventricular mass, as assessed by cardiac magnetic resonance imaging, and death or change in the physical-health composite score of the RAND 36-item health survey. Secondary outcomes included cognitive performance; self-reported depression; laboratory markers of nutrition, mineral metabolism, and anemia; blood pressure; and rates of hospitalization and of interventions related to vascular access. RESULTS: Patients in the frequent-hemodialysis group averaged 5.2 sessions per week; the weekly standard Kt/V(urea) (the product of the urea clearance and the duration of the dialysis session normalized to the volume of distribution of urea) was significantly higher in the frequent-hemodialysis group than in the conventional-hemodialysis group (3.54±0.56 vs. 2.49±0.27). Frequent hemodialysis was associated with significant benefits with respect to both coprimary composite outcomes (hazard ratio for death or increase in left ventricular mass, 0.61; 95% confidence interval [CI], 0.46 to 0.82; hazard ratio for death or a decrease in the physical-health composite score, 0.70; 95% CI, 0.53 to 0.92). Patients randomly assigned to frequent hemodialysis were more likely to undergo interventions related to vascular access than were patients assigned to conventional hemodialysis (hazard ratio, 1.71; 95% CI, 1.08 to 2.73). Frequent hemodialysis was associated with improved control of hypertension and hyperphosphatemia. There were no significant effects of frequent hemodialysis on cognitive performance, self-reported depression, serum albumin concentration, or use of erythropoiesis-stimulating agents. CONCLUSIONS: Frequent hemodialysis, as compared with conventional hemodialysis, was associated with favorable results with respect to the composite outcomes of death or change in left ventricular mass and death or change in a physical-health composite score but prompted more frequent interventions related to vascular access. (Funded by the National Institute of Diabetes and Digestive and Kidney Diseases and others; ClinicalTrials.gov number, NCT00264758.).

A global survey of the bacteria within earthworm nephridia.

Earthworms comprise 16 described families in the Crassiclitellata plus a few other minor groups. Microscopy studies of the early 20th century detected bacteria within the excretory organs, the nephridia, of species within a few of these families. More recent evidence for the consistent and specific association of bacteria with nephridia within the Lumbricidae has been well documented, but the presence and identity of nephridial bacteria among the rest of the Crassiclitellata families had not been explored. The study presented here aimed to identify members of Crassiclitellata families that harbor bacteria in their nephridia, and identify these bacteria based on 16S rRNA gene sequences. Eleven earthworm families were surveyed from countries of six continents, and two island nations. The results revealed members of four bacterial orders commonly occurred within nephridia of genera within nine Crassiclitellata families. Members of the bacterial phyla Bacteroidetes (order Sphingobacteriales), Betaproteobacteria (order Burkholderiales; family Comamonadaceae), and Alphaproteobacteria (orders Rhodospirillales and Rhizobiales) were detected in the nephridia of basal Crassiclitellata, as well as in derived families. Earthworm genera with meronephridia, multiple small nephridia per segment, lacked bacteria, whereas bacteria were often detected in holonephridia, single pairs of large nephridia with a distinct morphology and external excretory pore. The Acanthodrilidae members, a large derived family of earthworms, did not appear to possess nephridial bacteria regardless of nephridial form. Although earthworms from a variety of habitat types were sampled, there were no clear correlations of lifestyle with symbiont types, with the exception of the aquatic earthworms that contained bacteria unrelated to those in any other earthworms. The findings support an evolutionarily long association of bacteria within the Crassiclitellata, and suggest a contribution to nitrogen conservation for the earthworms.

Effects of frequent hemodialysis on measures of CKD mineral and bone disorder.

More frequent hemodialysis sessions and longer session lengths may offer improved phosphorus control. We analyzed data from the Frequent Hemodialysis Network Daily and Nocturnal Trials to examine the effects of treatment assignment on predialysis serum phosphorus and on prescribed dose of phosphorus binder, expressed relative to calcium carbonate on a weight basis. In the Daily Trial, with prescribed session lengths of 1.5-2.75 hours six times per week, assignment to frequent hemodialysis associated with both a 0.46 mg/dl decrease (95% confidence interval [95% CI], 0.13-0.78 mg/dl) in mean serum phosphorus and a 1.35 g/d reduction (95% CI, 0.20-2.50 g/d) in equivalent phosphorus binder dose at month 12 compared with assignment to conventional hemodialysis. In the Nocturnal Trial, with prescribed session lengths of 6-8 hours six times per week, assignment to frequent hemodialysis associated with a 1.24 mg/dl decrease (95% CI, 0.68-1.79 mg/dl) in mean serum phosphorus compared with assignment to conventional hemodialysis. Among patients assigned to the group receiving six sessions per week, 73% did not require phosphorus binders at month 12 compared with only 8% of patients assigned to sessions three times per week (P<0.001). At month 12, 42% of patients on nocturnal hemodialysis required the addition of phosphorus into the dialysate to prevent hypophosphatemia. Frequent hemodialysis did not have major effects on calcium or parathyroid hormone concentrations in either trial. In conclusion, frequent hemodialysis facilitates control of hyperphosphatemia and extended session lengths could allow more liberal diets and freedom from phosphorus binders.

Development of an Innovative Telerehabilitation System to Improve Handgrip Strength.

Handgrip strength is an essential function of the hand to perform day-to-day tasks. People lose grip strength due to various factors such as aging, diseases, and other medical conditions. According to neuroplastic and physiological principles, grip strength can be improved using goal-oriented tasks or exercises repeatedly and consistently. People often fail to adhere to repeated movements, including grip strength exercises. Studies have shown that game-based rehabilitation has improved exercise compliance and functional outcomes. This article explains the design and development of an affordable smartphone-based telerehabilitation system that includes an innovatively designed grip strength device (eGripper) and a phone application to play games.

Phylogenetic analysis of New Zealand earthworms (Oligochaeta: Megascolecidae) reveals ancient clades and cryptic taxonomic diversity.

We have constructed the first ever phylogeny for the New Zealand earthworm fauna (Megascolecinae and Acanthodrilinae) including representatives from other major continental regions. Bayesian and maximum likelihood phylogenetic trees were constructed from 427 base pairs from the mitochondrial large subunit (16S) rRNA gene and 661 base pairs from the nuclear large subunit (28S) rRNA gene. Within the Acanthodrilinae we were able to identify a number of well-supported clades that were restricted to continental landmasses. Estimates of nodal support for these major clades were generally high, but relationships among clades were poorly resolved. The phylogenetic analyses revealed several independent lineages in New Zealand, some of which had a comparable phylogenetic depth to monophyletic groups sampled from Madagascar, Africa, North America and Australia. These results are consistent with at least some of these clades having inhabited New Zealand since rifting from Gondwana in the Late Cretaceous. Within the New Zealand Acanthodrilinae, major clades tended to be restricted to specific regions of New Zealand, with the central North Island and Cook Strait representing major biogeographic boundaries. Our field surveys of New Zealand and subsequent identification has also revealed extensive cryptic taxonomic diversity with approximately 48 new species sampled in addition to the 199 species recognized by previous authors. Our results indicate that further survey and taxonomic work is required to establish a foundation for future biogeographic and ecological research on this vitally important component of the New Zealand biota.

Impact of sleep quality on cardiovascular outcomes in hemodialysis patients: results from the frequent hemodialysis network study.

BACKGROUND: Poor sleep quality is a common, persistent, and important problem to patients with end-stage renal disease (ESRD). This report examines whether sleep quality is associated with dialysis treatment factors and other modifiable clinical factors in a large group of hemodialysis (HD) patients. METHODS: Cross-sectional analyses were conducted on baseline data collected from participants in the Frequent Hemodialysis Network trials. Sleep quality was measured using the Medical Outcomes Study Sleep Problems Index II (SPI II), a 9-item measure of sleep quality with higher scores reflecting poorer sleep quality. RESULTS: The participants had an age of 51.2 ± 13.6 years, 61% were male, 38% were black, and 42% had diabetes. Higher pre-dialysis serum phosphorus (per 0.5 mg/ml) (OR 0.91; 95% CI 0.85, 0.96) and depression (OR 0.16; 95% CI 0.10, 0.25) were independently associated with decrements in sleep quality. There was also a difference in time to recovery from dialysis for the fourth versus the first SPI II quartile (5.1 h; p < 0.0001). CONCLUSION: These findings underscore the link between sleep and daytime function and suggest that improving sleep may provide an opportunity to improve outcomes in ESRD. Whether sleep problems may be improved by reduction of serum phosphorus or treatment of depression in the HD population merits further investigation.

An approach to giant neonatal hydrocolpos with normally sited anus - Diagnosis and management including a novel one stage operation.

INTRODUCTION: Giant non cloacal neonatal hydrocolpos is a rare entity where early recognition of the underlying cause and drainage of the distended vagina is paramount. Conventionally, immediate management has been by abdominal tube vaginostomy or, in urogenital sinus by vaginal clean intermittent catheterisation (CIC). Though effective in providing temporary relief, the abdominal tube vaginostomy is replete with complications, making it imperative to search for alternatives. Here we describe our method of emergent and definitive management of these children, where definitive repair, depending on the aetiology, has been made possible in a single stage. MATERIAL AND METHODS: Seven girls who presented primarily or with history of giant non cloacal neonatal hydrocolpos at two tertiary care centres from January 2013 to March 2021 were studied retrospectively. None had imperforate anus or a bulging hymenal membrane. Four had high vaginal atresia (VA) and three had Urogenital sinus (UGS), one with high confluence. Four girls (2 VA, 2 UGS) presented to us as neonates and were managed by emergency drainage procedures other than tube vaginostomy: CIC in one (low UGS) and a novel abdominoperineal U flap vaginoplasty in three (2 VA, 1 high confluence UGS) which was also the definitive procedure. This procedure involved fashioning an inferiorly based inverted U flap from the posterior wall of distended vagina which is tubularised and flipped down by an abdomino-perineal procedure. The girl with a low UGS later underwent a perineal procedure. Three presented elsewhere as neonates (2 VA, 1 UGS) and had abdominal tube vaginostomy as the emergency drainage procedure. This was complicated by tube dislodgement and pyocolpos till successful reconstruction was performed by us. Successful reconstructions included the abdominoperineal U flap vaginoplasty in one (VA) and a perineal procedure in another (low UGS). One girl with high VA had an inflamed vagina after tube vaginostomy, precluding the U flap vaginoplasty, and underwent abdominal vaginal mobilisation and pull through complicated by partial vaginal necrosis with septic complications. RESULTS: Reconstruction resulted in a patent and a good calibre neo-vagina in all at a mean follow up of 48 months. CONCLUSION: Immediate management of giant non cloacal neonatal hydrocolpos may either be vaginal CIC or a surgical procedure. The abdomino-perineal U flap vaginoplasty is a one stage neonatal procedure that provides effective drainage and forms a definitive neo-vagina. An abdominal tube vaginostomy can be avoided except where neonatal surgical expertise is unavailable.

Diversity and host specificity of the Verminephrobacter-earthworm symbiosis.

Symbiotic bacteria of the genus Verminephrobacter (Betaproteobacteria) were detected in the nephridia of 19 out of 23 investigated earthworm species (Oligochaeta: Lumbricidae) by 16S rRNA gene sequence analysis and fluorescence in situ hybridization (FISH). While all four Lumbricus species and three out of five Aporrectodea species were densely colonized by a mono-species culture of Verminephrobacter, other earthworm species contained mixed bacterial populations with varying proportions of Verminephrobacter; four species did not contain Verminephrobacter at all. The Verminephrobacter symbionts could be grouped into earthworm species-specific sequence clusters based on their 16S rRNA and RNA polymerase subunit B (rpoB) genes. Closely related host species harboured more closely related symbionts than did distantly related hosts. Co-diversification of the symbiotic partners could not be demonstrated unambiguously due to the poor resolution of the host phylogeny [based on histone H3 and cytochrome c oxidase subunit I (COI) gene sequence analyses]. However, there was a pattern of symbiont diversification within four groups of closely related hosts. The mean rate of symbiont 16S rRNA gene evolution was determined using a relaxed clock model, and the rate was calibrated with paleogeographical estimates of the time of origin of Lumbricid earthworms. The calibrated rates of symbiont 16S rRNA gene evolution are 0.012-0.026 substitutions per site per 50 million years and thus similar to rates reported from other symbiotic bacteria.

Investigation of food and environmental exposures relating to the epidemiology of Campylobacter coli in humans in Northwest England.

This study uses multilocus sequence typing (MLST) to investigate the epidemiology of Campylobacter coli in a continuous study of a population in Northwest England. All cases of Campylobacter identified in four Local Authorities (government administrative boundaries) between 2003 and 2006 were identified to species level and then typed, using MLST. Epidemiological information was collected for each of these cases, including food and recreational exposure variables, and the epidemiologies of C. jejuni and C. coli were compared using case-case methodology. Samples of surface water thought to represent possible points of exposure to the populations under study were also sampled, and campylobacters were typed with multilocus sequence typing. Patients with C. coli were more likely to be older and female than patients with C. jejuni. In logistic regression, C. coli infection was positively associated with patients eating undercooked eggs, eating out, and reporting problems with their water supply prior to illness. C. coli was less associated with consuming pork products. Most of the cases of C. coli yielded sequence types described elsewhere in both livestock and poultry, but several new sequence types were also identified in human cases and water samples. There was no overlap between types identified in humans and surface waters, and genetic analysis suggested three distinct clades but with several "intermediate" types from water that were convergent with the human clade. There is little evidence to suggest that epidemiological differences between human cases of C. coli and C. jejuni are a result of different food or behavioral exposures alone.

Management and outcome in dehisced exstrophy with a simplified bladder re-closure and further reconstruction.

INTRODUCTION: The traditionally accepted method of bladder re closure in dehisced exstrophy involves osteotomy assisted pubic bone approximation (PBA). Continent voluntary voiding is achieved in a small proportion of children sometimes after many operative procedures. We propose a simple yet reliable method of repair of the dehisced bladder using Rectus abdominis muscle flap (RAM) instead of PBA to bridge the inter-pubic gap, followed by concomitant or subsequent bladder augmentation (BA). AIM: The aim of this study is to assess the outcome of dehisced exstrophy, using a RAM flap assisted redo closure (without PBA) with concomitant or subsequent further reconstruction. MATERIALS AND METHODS: This is a retrospective analysis of children who presented with dehisced exstrophy after repair in other institutions and who have undergone redo repair using RAM flap in two tertiary care centers from 2001 to 2019. The outcome of the redo closure and subsequent or concomitant further reconstruction as regards dryness, stability of the upper tracts and resolution of vesico ureteric reflux (VUR) was studied. RESULTS: Fifty five children (34 boys) underwent redo exstrophy repair for dehisced exstrophy using the RAM flap. Epispadias repair was performed concomitantly in 31 boys. In 26 children (group1) of mean age 12 months further surgery was deferred while in 29 children (group 2) of mean age 69 months underwent concomitant BA. Nine group 1 children underwent BA subsequently. Ureteric reimplantation was done at the time of BA in 54 ureters, 40 into the bladder plate and rest into the bowel segment of BA. 22 ureters were not reimplanted. Bladder neck surgery including 18 bladder neck closure and Mitrofanoff port for Clean Intermittent catheterization (CIC) were done along with BA. The RAM assisted bladder closure was event free and none needed redo operation. 35/38 augmented children are dry on Mitrofanoff CIC and one unaugmented boy voids normally. The upper tracts remain stable on ultrasound and VUR has resolved in 67/76 ureters. At current follow up, after a mean period of 53 months eGFR was normal in all except 3 who had initially presented with severe hydro uretero nephrosis. CONCLUSION: We present a simple and reliable method of repair of dehisced exstrophy using RAM flap with the feasibility of concomitant BA. Dryness was achieved with stable upper tracts in 36/39 children, 27 of them with a single reconstructive attempt.

Transureteroureterostomy as an adjunctive antireflux procedure in children undergoing bladder augmentation for neurogenic bladder with major reflux.

INTRODUCTION: Transureteroureterostomy (TUU) provides urinary drainage of both renal systems to the bladder via a single ureter and is useful in selected situations of complex urological reconstructions. Herein we discuss its use, advantages and complications in children with neurogenic bladders and high-grade (4/5) reflux who have undergone augmentation cystoplasty. PATIENTS AND METHODS: Children with neurogenic bladder complicated by unilateral or bilateral high-grade vesicoureteric reflux (VUR), who underwent TUU along with augmentation cystoplasty (BA), were selected from two institutions. Eighteen children with an average age of 5 years at presentation were identified from a retrospective chart review. RESULTS: All had bilateral hydroureteronephrosis (HUN) of which there were 30 refluxing megaureters. While BA reduced bladder pressure, VUR was managed by refluxing to non-refluxing TUU in six cases with unilateral VUR and unilateral reimplantation with TUU to the reimplanted ureter in 12 cases of bilateral VUR, thus minimizing reimplantation to 12 of 30 ureters. The average time of follow-up was 51 months. Follow-up cystograms showed complete resolution of VUR in all. HUN improved/stabilized in all but one child. Serum creatinine remained normal in all but two cases. Other advantages of TUU include the use of the distal ureter as a catheterizable channel and ease of undiversion when the ureter has been diverted as a ureterostomy. An unusual complication of a TUU site stricture is discussed and the innovative technique of using a cecal patch to salvage the anastomosis is detailed. CONCLUSION: We conclude that a TUU is a safe and useful adjunctive procedure in children undergoing BA for neurogenic bladder with high-grade VUR, minimizing the need for ureteric reimplant in an unhealthy bladder.

Leukocyte chemotactic factor 2: A novel renal amyloid protein.

Renal amyloid deposits can often be seen in primary amyloidosis (immunoglobulin light chain disease) or in secondary forms such as reactive amyloidosis as well as in several hereditary forms where a variety of mutant proteins 'precipitate' as amyloid plaques. However, in rare cases, amyloidosis may be identified by renal biopsy, but no definitive diagnosis could be made. We have isolated amyloid fibrils from such a case in which the patient presented with nephrotic syndrome and subsequent azotemia requiring hemodialysis. Evaluation for amyloid deposition in other organ systems was negative and immunohistochemical analysis of the kidney deposits for known contributing proteins was unrevealing. Biochemical analysis of the fibrils identified a new amyloid subunit protein, leukocyte chemotactic factor 2, originally identified as a possible chemotactic and growth factor. A monoclonal antibody to this protein reacted specifically with the amyloid deposits in the glomeruli and interstitium by immunohistochemistry. This study emphasizes the importance of biochemical characterization of amyloid present in renal biopsies.

Living donor renal transplant recipients tolerate early removal of bladder catheters.

Recipients of living donor renal grafts enjoy numerous benefits compared with deceased donor kidney recipients. Bladder catheterization allows for the continuous determination of urinary output and, theoretically, may prevent urinary leaks. A series of 25 consecutive renal transplants was reviewed to evaluate the timing of removal of bladder catheters after transplantation. Removing urinary catheters as early as 24 h to 48 h post-transplant showed no increase in undesirable outcomes. More than 50% of the patients had invasive bladder catheters in place for only one or two days. Early removal was associated with a lower rate of urinary tract infections, decreased length of hospitalization and possibly less discomfort, in the absence of detrimental effects.

Cadaveric renal transplant recipients can safely tolerate removal of bladder catheters within 48 h of transplant.

The ideal time to remove urinary catheters after renal transplantation has not been thoroughly established. It remains unclear whether the anastomosis is actually protected with prolonged bladder catheterization. In addition, the incidence of urinary tract infections may increase with prolonged catheterization. A series of 57 consecutive deceased donor renal transplants was retrospectively reviewed for outcomes associated with duration of bladder catheterization. Removing urinary catheters within 48 h post-transplant showed no increase in undesirable outcomes, and very likely improved patient satisfaction.

Survival after ethylene glycol poisoning in a patient with an arterial pH of 6.58.

This ethylene glycol poisoning case had a blood pH of 6.58 and severe hypothermia (30.9 C). The patient received supportive care with dialysis and ethanol therapy. He survived in his premorbid state after 23 days in the hospital. A similar case survived ethylene glycol poisoning neurologicaly intact with an initial pH of 6.46. Although severe acidosis in the presence of serious illness is usually associated with a poor prognosis, our case emphasized the importance of aggressive supportive care and antidotal therapy for ethylene glycol poisoning even when there is a low pH.