What to Do With Renal Cysts in Children? Wang, Zhan Tao (Peter); Chan, Ernest Pang; Vanin Moreno, Nicolas ...
Urology (Ridgewood, N.J.),
June 2020, 2020-06-00, 20200601, Letnik:
140
Journal Article
Recenzirano
To explore the current practice patterns for the management of renal cysts internationally among pediatric urologists and nephrologists.
A survey composed of 21 questions and 4 clinical scenarios was ...distributed to pediatric urologists and nephrologists. Survey questions evaluated optimal imaging modality, management, and follow-up period. Interspecialty comparisons were made using chi-square analysis where appropriate.
A total of 183 respondents completed the survey (128 pediatric urologists, 37 pediatric nephrologists, and 19 other specialists). Most (57%) respondents agreed or strongly agreed with using an ultrasound based Bosniak classification to categorize renal cysts in children. The most commonly used follow-up intervals were 6-12 months for pediatric urologists and 1-2 years for pediatric nephrologists. Symptomatic mass effect (80.9%), gross hematuria (79.2%), or family history were the most common reasons for escalating surveillance. Pediatric nephrologists were more likely to increase follow-up with development of bilateral simple renal cysts (P = .008) or chronic kidney disease (P = .027) when compared to pediatric urologists. Laparoscopic marsupialization (39.4%) was the most common treatment for a simple renal cyst in a symptomatic child. Modified Bosniak III cysts had more heterogeneity in treatment based on the physician responses.
There is currently no consensus on the optimal protocol for the surveillance, imaging, or treatment of renal cysts in children. Most respondents agree that using an ultrasound-based Bosniak classification is reasonable. A call to action is therefore necessary for the development of registries and guidelines on the management of pediatric renal cysts and their associated malignancies.
Gene alterations are recognized as important events in acute myeloid leukemia (AML) progression. Studies on hematopoiesis of altered genes contribute to a better understanding on their roles in AML ...progression. Our previous work reported a DEAH box helicase 15 (DHX15) R222G mutation in AML patients, and we showed DHX15 overexpression is associated with poor prognosis in AML patients. In this work, we further study the role of dhx15 in zebrafish developmental hematopoiesis by generating dhx15−/− zebrafish using transcription activator‐like effector nuclease technology. Whole‐mount in situ hybridization (WISH) analysis showed hematopoietic stem/progenitor cells were dramatically perturbed when dhx15 was deleted. Immunofluorescence staining indicated inhibited hematopoietic stem/progenitor cell (HSPC) proliferation instead of accelerated apoptosis were detected in dhx15−/− zebrafish. Furthermore, our data showed that HSPC defect is mediated through the unfolded protein response (UPR) pathway. DHX15 R222G mutation, a recurrent mutation identified in AML patients, displayed a compromised function in restoring HSPC failure in dhx15−/−; Tg (hsp: DHX15 R222G) zebrafish. Collectively, this work revealed a vital role of dhx15 in the maintenance of definitive hematopoiesis in zebrafish through the unfolded protein respone pathway. The study of DHX15 and DHX15 R222G mutation could hold clinical significance for evaluating prognosis of AML patients with aberrant DHX15 expression.
Dhx15 is essential for hematopoietic stem/progenitor cell (HSPC) development in zebrafish; HSPC failure is mediated by the unfolded protein response (UPR) pathway in dhx15−/− zebrafish.
Abstract Glioblastoma multiforme (GBM) has a poor prognosis despite maximal multimodal therapy. Biomarkers of relevance to prognosis which may also identify treatment targets are needed. A few ...hundred genetic and molecular predictors have been implicated in the literature, however with the exception of IDH1 and O6-MGMT, there is uncertainty regarding their true prognostic relevance. This study analyses reported genetic and molecular predictors of prognosis in GBM. For each, its relationship with univariate overall survival in adults with GBM is described. A systematic search of MEDLINE (1998–July 2010) was performed. Eligible papers studied the effect of any genetic or molecular marker on univariate overall survival in adult patients with histologically diagnosed GBM. Primary outcomes were median survival difference in months and univariate hazard ratios. Analyses included converting 126 Kaplan–Meier curves and 27 raw data sets into primary outcomes. Seventy-four random effects meta-analyses were performed on 39 unique genetic or molecular factors. Objective criteria were designed to classify factors into the categories of clearly prognostic, weakly prognostic, non-prognostic and promising. Included were 304 publications and 174 studies involving 14,678 unique patients from 33 countries. We identified 422 reported genetic and molecular predictors, of which 52 had ⩾2 studies. IDH1 mutation and O6-MGMT were classified as clearly prognostic, validating the methodology. High Ki-67/MIB-1 and loss of heterozygosity of chromosome 10/10q were classified as weakly prognostic. Four factors were classified as non-prognostic and 13 factors were classified as promising and worthy of additional investigation. Funnel plot analysis did not identify any evidence of publication bias. This study demonstrates a novel literature and meta-analytical based approach to maximise the value that can be derived from the plethora of literature reports of molecular and genetic factors in GBM. Caution is advised in over-interpreting the results due to study limitations. Further research to develop this methodology and improvements in study reporting are suggested.
Background
The incidence of nephrolithiasis in children and adolescents is increasing and appears to double every 10 years. The most important role of the pediatric nephrologist is to diagnose and ...modify various metabolic and non-metabolic risk factors, as well as prevent long-term complications especially in the case of recurrent nephrolithiasis.
Objective
The purpose of this review is to summarize the existing literature on the etiology and management of pediatric nephrolithiasis.
Results
The incidence of kidney stones is increasing; dietary and environmental factors are probably the main causes for this increased incidence. In most pediatric patients, the etiology for the kidney stones can be identified. Metabolic factors, such as hypercalciuria and hypocitraturia, urinary tract infection, and urinary stasis, constitute leading causes. Herein, we review the etiologies, diagnostic work-up, and treatment options for the most prevalent causes of kidney stones. The detrimental effects of excessive dietary sodium, reduced fluid intake, and the benefits of plant-based over animal-based protein consumption on urinary crystal formation are discussed. We also review the long-term complications.
Conclusions
Pediatric nephrologists have an important role in the diagnostic work-up and prevention of recurring nephrolithiasis.
Studies indicate that learning surgical skills on low-fidelity models is equally beneficial to learning on high-fidelity models in terms of skills retention and transfer. However, it is unclear how ...low-fidelity simulation training impacts retention and transfer in novice learners, particularly on complex surgical tasks that incorporate multiple challenging skills. This study explores the capacity of complete novices to learn and transfer complex surgical skills from a low-fidelity model to a high-fidelity simulation after a delay.
Task-naïve medical and nonmedical undergraduate students (n = 62) participated in a three-phase prospective double-arm randomized (2:1) experimental study. Participants completed two skills training sessions (end-to-side anastomosis) on a low-fidelity bench model. After a 4-week delay, participants completed the task again either using the low-fidelity model or a high-fidelity model (cadaver) and were assessed using a validated checklist.
There was a significant time × fidelity group interaction (P = 0.004). Simple effects analysis indicated the high-fidelity group (Mdiff = 4.18, P < 0.001) performed significantly worse (P = 0.003) in phase 3 relative to phase 2 compared to the low-fidelity group (Mdiff = 0.75, P = 0.39). Post hoc logistic regression analysis indicated that radial suturing technique and economy of motion skills were less likely to be completed correctly for those in the high-fidelity group.
These findings suggest that for novice populations, relying on low-fidelity simulation training as a source of teaching complex skills may not provide a reliable transfer to high-fidelity models and in turn clinical settings.
Background
Galloway-Mowat syndrome (GAMOS) (OMIM #251300) is a severe autosomal recessive disease characterized by the combination of early-onset steroid-resistant nephrotic syndrome (SRNS) and ...microcephaly with brain anomalies caused by
WDR73
as well as
OSGEP
,
TP53RK
,
TPRKB
, or
LAGE3
mutations.
Objective
We report on the hitherto undescribed urological and nephrological complications of the homozygous c.974G>A (p.Arg325Gln)
OSGEP
mutations in a 7-year-old Caucasian girl.
Case diagnosis
The patient came to the attention of pediatric nephrology at the age of 3 years and 11 months, when she presented with status epilepticus due to profound hypomagnesemia (0.31 mmol/L, normal 0.65–1.05). A 24-h urine demonstrated a magnesium loss of 0.6 mmol/kg/day with associated proteinuria suggesting renal tubulopathy. Subsequently, she developed recurrent urinary tract infections (UTIs) and was diagnosed with neurogenic bladder dysfunction. The patient continued to have UTIs associated with seizures and sequential cultures growing multi-drug-resistant organisms despite of antibiotic prophylaxis. In addition, the proteinuria (median microalbumin/creatinine ratio 647 mg/mmol) increased, and she developed partial Fanconi syndrome. At age 7, she developed a large bladder calculus (3.3 × 3.2 cm) and three left non-obstructing renal calculi associated with elevated urinary cystine, hypercalciuria, and ongoing hypomagnesemia and required surgical intervention. Glomerular filtration rate (GFR) remained normal and she never developed frank nephrotic syndrome (average albumin 31 g/L).
Conclusions
It is unclear if patients with
OSGEP
mutations with tubular symptoms rather than nephrotic syndrome should be considered a different entity. Nephrological and urological complications of
OSGEP
mutations can be challenging and require a multidisciplinary approach.
The objective of this study was to examine the association between learner personality and capacity to be trained (i.e., performance improvement) on a surgical task, and how instructor perceptions of ...the learners’ capacity to be trained interact with learner personality and performance during training and feedback. There is meaningful heterogeneity in the degree of learner surgical skills acquisition despite receiving the same amount of training. While learner personality may independently contribute to skill acquisition, the instructor-learner feedback process is also important to consider. To better understand this interpersonal relationship, it is necessary to also consider instructor factors (i.e., perceptions), and how this may contribute to learner variability in skills training.
This exploratory study employed a prospective two-phase design. Medical and non-medical undergraduate students (N = 62) completed measures of personality and participated in two 20-minute training sessions with expert feedback 2 weeks apart, performing an end-to-side anastomosis on a low-fidelity model. Learner performance and instructors’ perceptions of a learner's capacity to be trained were assessed.
Sixty-two medical and non-medical undergraduate students.
There was a significant interaction between learner Extraversion and instructor's perceptions of learner capacity to be trained. Higher learner Extraversion was only associated with an increase in performance improvement for those who were considered trainable (OR = 4.83, p = 0.017). Post hoc analysis revealed a significant difference in the amount of feedback provided to participants who were considered trainable (M = 9.45) versus not trainable (M = 16.48).
This study highlights the importance of both individual learner factors and instructor perceptions on surgical skill acquisition.
Demonstrated competence through frequent assessment is an expected goal for progressive development in competency-based medical education curricula. The Objective Structured Assessment of Technical ...Skill (OSATS) is considered a valid method of formative assessment, but in few instances have standards been set for determining competence. The present study used borderline regression methods to examine standard setting of performance on a complex technical task with novices assessed using an OSATS checklist.
This was a single institution prospective single arm experimental design study. Participants were 58 non-medical undergraduate students with no previous surgical experience, who observed a computer-based training module on end-to-side vascular anastomosis. Subsequently, participants were provided two 20-minute training sessions, two weeks apart where they received expert feedback whilst performing the task on a low-fidelity model. After each training session, participants completed the task unaided. Sessions were recorded and assessed using an OSATS checklist retrospectively by experts.
Paired t-test analyses indicate that for both the checklist total score (t(52) = 8.05, p < 0.001) and the global rating score (t(53) = 8.15, p < 0.001), individuals performed significantly better in Phase 2. Borderline regression analyses indicated that in Phase 1 (R2 = .60) and Phase 2 (R2 = .75), the OSATS checklist could adequately capture variation in performance in novices. Further, the checklist could reliably classify novices at three of the five global rating performance levels. Pass rates determined by regression equations improved from Phase 1 to Phase 2 on all global rating levels.
With the increasing focus on competency-based medical education, it is imperative that training programs have the capacity to accurately assess outcomes and set minimum performance standards. Borderline regression methods can accurately differentiate novice learners of varying performance levels before and after training on a complex technical skill task using an OSATS checklist.
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