OBJECTIVE: To develop consensus on diagnostic criteria for LUMBAR syndrome, the association of segmental infantile hemangiomas that affect the Lower body with Urogenital anomalies, Ulceration, spinal cord Malformations, Bony defects, Anorectal malformations, Arterial anomalies and/or Renal anomalies.
METHODS: These diagnostic criteria were developed by an expert multidisciplinary and multi-institutional team based on analysis of peer-reviewed data, followed by electronic-Delphi consensus of a panel of 61 international pediatric specialists.
RESULTS: After 2 Delphi rounds, a 92% or higher level of agreement was reached for each Delphi statement. 98% of panelists agreed with the diagnostic criteria, and 100% agreed the criteria would be useful in clinical practice. The diagnosis of LUMBAR requires the presence of a segmental, or patterned, infantile hemangioma of the lumbosacral, sacrococcygeal, or pelvic cutaneous regions plus one additional criterion of the urogenital, spinal, bony, anorectal, arterial, or renal organ systems.
CONCLUSIONS: These diagnostic criteria will enhance clinical care by improving screening, detection, and overall awareness of this poorly understood neurocutaneous disorder. The criteria can be utilized by a wide variety of pediatric subspecialists. In addition, formal criteria will improve phenotypic uniformity among LUMBAR syndrome cohorts and a patient registry, allowing investigators to assess clinical features, long-term outcomes, and results of genetic sequencing in a standardized manner. Finally, these criteria will serve as a starting point for prospective studies to establish formal screening and management guidelines.

女性性功能障碍（FSD）是全球的重要公共卫生问题。鉴于目前国内尚无针对FSD诊治的标准参考，中国整形美容协会科技创新与器官整复分会组织国内性医学领域、妇产科学领域、康复治疗领域、心理卫生领域以及中医领域的专家，结合国内外FSD研究现状制定本共识，以期为FSD临床诊治提供参考。本共识的制定严格遵循指南共识制定的通用原则，并按照循证医学原则划分证据等级并给出推荐等级。本共识定义了FSD，并对FSD的流行病学进行了概述，明确了性反应周期的四阶段学说以及FSD的常见病因与高危因素。本共识参考国际上普遍采用的分类原则对FSD进行了分类，包括：性欲减退功能障碍、性唤起功能障碍、性高潮功能障碍、生殖器或盆腔疼痛和插入障碍、与盆底功能障碍性疾病有关的性功能障碍、其他特指及未特指的性功能障碍。本共识对FSD的诊断和治疗提出了原则性的指导。FSD的诊断要重视病史采集，并合理应用性功能评估量表或问卷；体格检查尤其是生殖系统专科检查是必不可少的，必要时要进行辅助检查。FSD的治疗包括一般干预、心理干预（包含性感集中疗法、认知行为疗法、正念疗法等）、药物治疗（其中的激素治疗包含雌激素治疗和雄激素治疗）、阴道润滑剂和保湿剂、自我性刺激训练、盆底物理疗法（包含盆底肌训练、阴道扩张、手法按摩、电刺激和生物反馈等）、原发疾病的治疗、中医药治疗。.

Kidney dysplasia is one of the most frequent causes of chronic kidney failure in children. While dysplasia is a histological diagnosis, the term \'kidney dysplasia\' is frequently used in daily clinical life without histopathological confirmation. Clinical parameters of kidney dysplasia have not been clearly defined, leading to imprecise communication amongst healthcare professionals and patients. This lack of consensus hampers precise disease understanding and the development of specific therapies. Based on a structured literature search, we here suggest a common basis for clinical, imaging, genetic, pathological and basic science aspects of non-obstructive kidney dysplasia associated with functional kidney impairment. We propose to accept hallmark sonographic findings as surrogate parameters defining a clinical diagnosis of dysplastic kidneys. We suggest differentiated clinical follow-up plans for children with kidney dysplasia and summarize established monogenic causes for non-obstructive kidney dysplasia. Finally, we point out and discuss research gaps in the field.

In recent years, several studies have been published on the prognosis of children with congenital solitary kidney (CSK), with controversial results, and a worldwide consensus on management and follow-up is lacking. In this consensus statement, the Italian Society of Pediatric Nephrology summarizes the current knowledge on CSK and presents recommendations for its management, including diagnostic approach, nutritional and lifestyle habits, and follow-up. We recommend that any antenatal suspicion/diagnosis of CSK be confirmed by neonatal ultrasound (US), avoiding the routine use of further imaging if no other anomalies of kidney/urinary tract are detected. A CSK without additional abnormalities is expected to undergo compensatory enlargement, which should be assessed by US. We recommend that urinalysis, but not blood tests or genetic analysis, be routinely performed at diagnosis in infants and children showing compensatory enlargement of the CSK. Extrarenal malformations should be searched for, particularly genital tract malformations in females. An excessive protein and salt intake should be avoided, while sport participation should not be restricted. We recommend a lifelong follow-up, which should be tailored on risk stratification, as follows: low risk: CSK with compensatory enlargement, medium risk: CSK without compensatory enlargement and/or additional CAKUT, and high risk: decreased GFR and/or proteinuria, and/or hypertension. We recommend that in children at low-risk periodic US, urinalysis and BP measurement be performed; in those at medium risk, we recommend that serum creatinine also be measured; in high-risk children, the schedule has to be tailored according to kidney function and clinical data.

Fetal lower urinary tract obstruction (LUTO) is associated with high mortality and postnatal morbidity caused by lung hypoplasia and impaired kidney function. Specific diagnostic features that can guide clinical approach and decisions are lacking; thus, the European Reference Network for Rare Kidney Diseases established a work group to develop recommendations regarding the clinical definition, diagnosis and management of prenatally detected LUTO. The work group recommends the use of antero-posterior diameter of renal pelvis as the most reliable parameter for suspecting obstructive uropathies and for suspecting prenatal LUTO in the presence of fetal megacystis. Regarding prenatal and postnatal prognosis of fetuses with LUTO, the risk of fetal and neonatal death depends on the presence of oligohydramnios or anhydramnios before 20 weeks\' gestation, whereas the risk of kidney replacement therapy cannot be reliably foreseen before birth. Parents of fetuses with LUTO must be referred to a tertiary obstetric centre with multidisciplinary expertise in prenatal and postnatal management of obstructive uropathies, and vesico-amniotic shunt placement should be offered in selected instances, as it increases perinatal survival of fetuses with LUTO.

OBJECTIVE: To develop imaging guidelines for the MR work-up of female genital tract congenital anomalies (FGTCA).
METHODS: These guidelines were prepared based on a questionnaire sent to all members of the European Society of Urogenital Radiology (ESUR) Female Pelvic Imaging Working Group (FPI-WG), critical review of the literature and expert consensus decision.
RESULTS: The returned questionnaires from 17 different institutions have shown reasonable homogeneity of practice. Recommendations with focus on patient preparation and MR protocol are proposed, as these are key to optimised examinations. Details on MR sequences and planning of uterus-orientated sequences are provided.
CONCLUSIONS: The multiplanar capabilities and soft tissue resolution of MRI provide superb characterisation of the wide spectrum of findings in FGTCA. A standardised imaging protocol and method of reporting ensures that the salient features are recognised, contributing to a correct diagnosis and classification of FGTCA, associated anomalies and complications. These imaging guidelines are based on current practice among expert radiologists in the field and incorporate up to date information regarding MR protocols and essentials of recently published classification systems.
CONCLUSIONS: • MRI allows comprehensive evaluation of female genital tract congenital anomalies, in a single examination. • A dedicated MRI protocol comprises uterus-orientated sequences and vaginal and renal evaluation. • Integration of classification systems and structured reporting helps in successful communication of the imaging findings.

We developed treatment guidelines (TGs) for appropriate transitional care of the genitourinary system in patients with persistent cloaca (PC), cloacal exstrophy (CE), or Mayer-Rokitansky-Küster-Häuser syndrome (MRKH). These TGs are in accordance with the Medical Information Network Distribution Service (Minds), published in 2014 in Japan. Clinical questions (CQs) concerning treatment outcomes of the genitourinary system, pregnancy and delivery, and quality of life in adulthood were prepared as six themes for PC and CE and five themes for MRKH. We were able to publish statements on chronic renal dysfunction, hydrometrocolpos, and pregnancy, based on four CQs about PC, four about CE, and two about MRKH, respectively. However, due to the paucity of proper manuscripts, we were unable to make conclusions about the correct timing and method of vaginoplasty for patients with PC, CE, and MRKH or the usefulness of early bladder closure for patients with CE. These TGs may help clarify the current treatments for PC, CE, and MRKH in childhood, which have been carried out on an institutional basis. To improve clinical outcomes, systematic clinical trials revealing comprehensive clinical data of the urinary and reproductive systems, especially the length of the common channel in PC, are essential.

A multidisciplinary urinary tract dilation (UTD) classification system was published in 2014 to standardize definitions and renal/bladder ultrasound image interpretation.
To evaluate intra- and inter-rater reliability of this system on postnatal RBUS.
Renal/bladder US of 60 infants (<12 months) with urinary tract dilation were anonymized, retrospectively reviewed and scored twice using the UTD classification system by a pediatric urologist and four pediatric radiologists. Exams included supine and prone images of each kidney. Raters recorded the anterior posterior renal pelvis diameter in each position; and when present calyceal dilation (central and peripheral), ureteral dilation, parenchymal and bladder abnormalities. A UTD score was given to each kidney based on these components.
Intraclass correlation (ICC) of anterior posterior renal pelvis diameter measures was 0.99 (P<0.001). Intra-rater reliability for the anterior posterior renal pelvis diameter of each kidney was high, with ICC >0.95 (P<0.001). Inter-rater kappa values for UTD scores of both kidneys ranged from 0.60 to 0.77 (P <0.001). Intra-rater kappa values for UTD scores of both kidneys ranged from 0.74 to 0.92 (P <0.001). Of the six categories comprising the UTD score, discrepancy between raters was highest for interpretation of central and peripheral calyceal dilation.
Present inter- and intra-rater reliability findings were similar to those previously reported for grading systems for urinary tract dilation. Across these studies, kappa values are generally lower than the 0.8 cut-off advocated for medical measures. Here, calyceal dilation commonly determined the kidney UTD score and was also the source of greatest discrepancy between raters. Improving consistency of calyceal dilation interpretation may improve UTD consensus score reliability.

暂无摘要。

Shockwave lithotripsy (SWL) is a widely utilized form of treatment for urolithiasis. There are new evidence-based recommendations regarding pre-SWL patient work-up and the performance of SWL. The purpose of this study is to determine practice patterns for SWL and to determine if regional variation exists between Canada and the United States.
A 19-question survey was prepared. Canadian urologists were surveyed through e-mail correspondence. In the United States, members of the Endourologic Society and members of two large stone management groups were surveyed. Canadian and American results were compared using the chi-square and Fisher\'s exact tests.
Ninety-four Canadian urologists and 187 U.S. urologists completed the survey. Practice patterns differed between countries. Intravenous sedation was more commonly used in Canada (Canada 94.7% vs United States 17.9%, p < 0.001); routine antibiotics were more commonly given in United States (Canada 2.1% vs United States 78.1%, p < 0.001); a shock rate of 2 Hz was more common in Canada (Canada 76.6% vs United States 16.2%, p < 0.00001); rate of discontinuing ASA for renal and ureteral stone treatment was higher in the United States (renal Canada 88.3% vs United States 95.7%, p < 0.02; ureteral Canada 62.4% vs 90.3%, p < 0.0001); and ureteral stents were more commonly used if treating a large stone or patients with solitary kidneys in the United States (large stones Canada 58.2% vs United States 88.8%, p = 0.0001; solitary kidney Canada 50.6% vs 66.3%, p = 0.02).
This study highlights the absence of standardization of SWL. Significant regional differences exist in practice patterns and performance of SWL between Canadian and American urologists.