Sex difference and risk factors in burden of urogenital congenital anomalies from 1990 to 2019.

Urogenital congenital anomalies (UCAs) is defined as "any live-birth with a urinary or genital condition" and affects millions of men and women worldwide. However, sex differences and related environmental risk factors in UCAs burden on a global scale have not been assessed. Using data from the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2019, we estimated prevalence, incidence, mortality and disability-adjusted life years (DALYs) of UCAs from 1990 to 2019 by sex, region, and socio-demographic Index (SDI) in 204 countries and territories. The disease burden of UCAs was also estimated attributable to each risk factor were estimated according to risk exposure. In 2019, UCAs caused 10,200 all-ages deaths (95% UI 7550-13,400). The combined global incidence rate was 8.38 per 1000 (95% UI 5.88-12.0) live births. The ASIR increased slightly, while the ASDR decreased from 1990 to 2019.The UCAs burden varies greatly depending on the development level and geographical location. The UCAs burden was significantly higher in men than in women, and the sex differences showed an enlarging trend. Health risks and issues, including pollution, child and maternal malnutrition, diet habits, unsafe sanitation and water source, were detected to be positively related to UCAs burden. Albeit the age-standardised prevalence, mortality, incidence, and DALYs of UCAs have decreased, they still cause a public health challenge worldwide. The high deaths and DALYs rates in low and low-middle SDI countries highlight the urgent need for improved preventive, diagnostic, and therapeutic measures. Global strategies for enhancing water safety, reducing pollution, and healthy diets are crucial steps in reducing the burden of UCAs.

Creation of a Pilot Surgical Program for the Comprehensive Management of Patients with Congenital Urological Malformations / Creación de un programa quirúrgico piloto para el manejo integral de pacientes con malformaciones urológicas congénitas

Objectives Congenital malformations constitute the first cause of morbidity and mortality in childhood in Latin America. That is why, since 2001, a surveillance system for congenital malformations has been implemented in Bogota - Colombia. However, despite the increase in detection, an impact on treatment has not been achieved. The present study describes our experience with a novel social program focused on congenital urologic disorders. Methods The present manuscript is a retrospective observational study. We reviewed two national databases containing patients with congenital malformations. Patients were actively contacted to verify the status of the malformations. Children in whom the malformation was confirmed were offered a free consultation with a multidisciplinary group. After screening for surgical indications, patients were scheduled for surgery. Results Between November 2018 and December 2019, 60 patients were identified. In total 44, attended the consultation; the remaining did not attend due to financial or travel limitations. The most common condition assessed was hypospadias. In total, 29 patients underwent surgery. The total cost of care was of US$ 5,800. Conclusions Active search improves attention times and reduces the burden of disease. The limitations to be resolved include optimizing the transportation of patients and their families, which is a frequent limitation to access health care.

Objetivos Las malformaciones congénitas corresponden a la principal causa de morbimortalidad en la infancia en América Latina, motivo por el cual desde el 2001 se viene implementando un sistema de vigilancia epidemiológica de malformaciones congénitas en Bogotá, Colombia. Sin embargo, a pesar del aumento en la cobertura del reporte obligatorio, no se ha logrado un impacto sobre su tratamiento. Este estudio busca mostrar nuestra experiencia con un programa integral de pacientes con malformaciones urológicas congénitas. Métodos El presente es un estudio observacional retrospectivo. Los menores con malformaciones congénitas fueron identificados en dos bases de datos nacionales que incluyen pacientes con malformaciones congénitas. Los pacientes reportados fueron contactados telefónicamente para verificar el estado actual de la malformación. A aquellos niños en quienes se les confirmó la malformación, se les ofreció de manera gratuita una consulta con un grupo multidisciplinario. Una vez confirmadas las indicaciones quirúrgicas, fueron llevados a cirugía. Resultados Se identificaron 60 pacientes entre noviembre del 2018 y diciembre de 2019. De los pacientes identificados, 44 acudieron a consulta. Los demás no asistieron por limitaciones económicas. La principal condición valorada fue hipospadias. En total, 29 pacientes fueron llevados a cirugía. El costo total de la atención de estos pacientes fue de 22 millones de pesos colombianos. Conclusiones La búsqueda activa mejora los tiempos de atención y reduce la carga de la enfermedad. Una de las limitaciones aun por resolver es optimizar el transporte de los pacientes y sus familias, que resulta una limitación frecuente para el acceso a la salud.

Prevalence of T-shaped uterus among fertile women based on ESHRE/ESGE and Congenital Uterine Malformation by Experts (CUME) criteria.

RESEARCH QUESTION: What is the prevalence of T-shaped uteri among fertile women based on ESHRE/ESGE and Congenital Uterine Malformation by Experts (CUME) criteria? DESIGN: A prospective cohort study of 258 women of reproductive age with a history of at least one natural pregnancy resulting in live birth. Participants were recruited from the family planning clinic between January 2018 and March 2020. The ESHRE/ESGE classification of congenital anomalies of the female genital tract was used for describing abnormal findings. CUME criteria were also used for diagnosing T-shaped uterus. Uterine cavity volume was measured. RESULTS: Mean age of participants was 35.4 ± 6.2 years. Participants were diagnosed with the following: congenital uterine abnormality (n = 9 [3.6%]); partial septate uterus (n = 5 [2.0%]) and hemiuterus (n = 2 [0.8%]). Two women (0.8%) were diagnosed with T-shaped uterus and borderline T-shaped uterus based on the ESHRE/ESGE criteria and CUME. Mean lateral indentation angle, lateral indentation depth and T-angle were 156.2° ± 9.53°, 2.85 ± 0.93 mm and 73.3° ± 9.85° in patients with normal uterine cavity. In patients with T-shaped and borderline T-shaped uteri, respective figures were 115° versus 121°, 10 mm versus 7.6 mm and 27.5° versus 70°. Median volume of the uterine cavity in patients with normal uterine cavity and T-shaped uterus was 3.71 ml (minimum 2.0 to maximum 9.03 ml, interquartile range 1.93) and 3.2 ml (2.9 and 3.62 ml), respectively. CONCLUSIONS: The prevalence of T-shaped uteri in fertile women is low, which corresponds to previous reports of women with poor reproductive history.

Challenges of access to kidney care for children in low-resource settings.

Kidney disease is a global public health concern across the age spectrum, including in children. However, our understanding of the true burden of kidney disease in low-resource areas is often hampered by a lack of disease awareness and access to diagnosis. Chronic kidney disease (CKD) in low-resource settings poses multiple challenges, including late diagnosis, the need for ongoing access to care and the frequent unavailability of costly therapies such as dialysis and transplantation. Moreover, children in such settings are at particular risk of acute kidney injury (AKI) owing to preventable and/or reversible causes - many children likely die from potentially reversible kidney disease because they lack access to appropriate care. Acute peritoneal dialysis (PD) is an important low-cost treatment option. Initiatives, such as the Saving Young Lives programme, to train local medical staff from low-resource areas to provide care for AKI, including acute PD, have already saved hundreds of children. Future priorities include capacity building for both educational purposes and to provide further resources for AKI management. As local knowledge and confidence increase, CKD management strategies should also develop. Increased awareness and advocacy at both the local government and international levels will be required to continue to improve the diagnosis and treatment of AKI and CKD in children worldwide.

Interkinetic nuclear movements promote apical expansion in pseudostratified epithelia at the expense of apicobasal elongation.

Pseudostratified epithelia (PSE) are a common type of columnar epithelia found in a wealth of embryonic and adult tissues such as ectodermal placodes, the trachea, the ureter, the gut and the neuroepithelium. PSE are characterized by the choreographed displacement of cells' nuclei along the apicobasal axis according to phases of their cell cycle. Such movements, called interkinetic movements (INM), have been proposed to influence tissue expansion and shape and suggested as culprit in several congenital diseases such as CAKUT (Congenital anomalies of kidney and urinary tract) and esophageal atresia. INM rely on cytoskeleton dynamics just as adhesion, contractility and mitosis do. Therefore, long term impairment of INM without affecting proliferation and adhesion is currently technically unachievable. Here we bypassed this hurdle by generating a 2D agent-based model of a proliferating PSE and compared its output to the growth of the chick neuroepithelium to assess the interplay between INM and these other important cell processes during growth of a PSE. We found that INM directly generates apical expansion and apical nuclear crowding. In addition, our data strongly suggest that apicobasal elongation of cells is not an emerging property of a proliferative PSE but rather requires a specific elongation program. We then discuss how such program might functionally link INM, tissue growth and differentiation.

Septate uterus according to ESHRE/ESGE, ASRM and CUME definitions: association with infertility and miscarriage, cost and warnings for women and healthcare systems.

OBJECTIVES: To estimate the differences in frequency of diagnosis of septate uterus using three different definitions and determine whether these differences are significant in clinical practice, and to examine the association between diagnosis of septate uterus, using each of the three definitions, and infertility and/or previous miscarriage as well as the cost of allocation to surgery. METHODS: This was a secondary analysis of data from a prospective study of 261 consecutive women of reproductive age attending a private clinic focused on the diagnosis and treatment of congenital uterine malformations. Reanalysis of the datasets was performed according to three different means of defining septate uterus: following the recommendations of the American Society for Reproductive Medicine (ASRM), a 2016 update of those of the American Fertility Society from 1988 (ASRM-2016: internal fundal indentation depth ≥ 1.5 cm, angle of internal indentation < 90° and external indentation depth < 1 cm); following the recommendations of the European Society of Human Reproduction and Embryology/European Society for Gynaecological Endoscopy (ESHRE/ESGE), published in 2013 and reaffirmed in 2016 (ESHRE/ESGE-2016: internal fundal/uterine indentation depth > 50% of uterine-wall thickness and external indentation depth < 50% of uterine-wall thickness, with uterine-wall thickness measured above interostial/intercornual line); and using a definition published last year which was based on the decision made most often by a group of experts (Congenital Uterine Malformation by Experts; CUME) (CUME-2018: internal fundal indentation depth ≥ 1 cm and external fundal indentation depth < 1 cm). We compared the rate of diagnosis of septate uterus using each of these three definitions and, for each, we estimated the association between the diagnosis and infertility and/or previous miscarriage, and anticipated the costs associated with their implementation using a guesstimation method. RESULTS: Although 32.6% (85/261) of the subjects met the criteria for one of the three definitions of septate uterus, only 2.7% (7/261) of them were defined as having septate uterus according to all three definitions. We diagnosed significantly more cases of septate uterus using ESHRE/ESGE-2016 than using ASRM-2016 (31% vs 5%, relative risk (RR) = 6.7, P < 0.0001) or CUME-2018 (31% vs 12%, RR = 2.6, P < 0.0001) criteria. We also observed frequent cases that could not be classified definitively by ASRM-2016 (gray zone: neither normal/arcuate nor septate; 6.5%). There were no significant differences (P > 0.05) in the prevalence of septate uterus in women with vs those without infertility according to ASRM-2016 (5% vs 4%), ESHRE/ESGE-2016 (35% vs 28%) or CUME-2018 (11% vs 12%). Septate uterus was diagnosed significantly more frequently in women with vs those without previous miscarriage according to ASRM-2016 (11% vs 3%; P = 0.04) and CUME-2018 (22 vs 10%; P = 0.04), but not according to ESHRE/ESGE-2016 (42% vs 28%; P = 0.8) criteria. Our calculations showed that global costs to the healthcare system would be highly dependent on the criteria used in the clinical setting to define septate uterus, with the costs associated with the ESHRE/ESGE-2016 definition potentially being an extra US$ 100-200 billion over 5 years in comparison to ASRM-2016 and CUME-2018 definitions. CONCLUSIONS: The prevalence of septate uterus according to ESHRE/ESGE-2016, ASRM-2016 and CUME-2018 definitions differs considerably. An important limitation of the ASRM classification, which needs to be addressed, is the high proportion of unclassifiable cases originally named, by us, the 'gray zone'. The high rate of overdiagnosis of septate uterus according to ESHRE/ESGE-2016 may lead to unnecessary surgery and therefore unnecessary risk in these women and may impose a considerable financial burden on healthcare systems. Efforts to define clinically meaningful and universally applicable criteria for the diagnosis of septate uterus should be encouraged. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Útero septo según las definiciones de ESHRE/ESGE, ASRM y CUME: la relación con la infertilidad y el aborto espontáneo, el costo y advertencias para las mujeres y los sistemas de salud OBJETIVO: Evaluar el rendimiento de la velocidad sistólica máxima de la arteria cerebral media fetal (MCA-PSV, por sus siglas en inglés) ≥1,5 múltiplos de la mediana (MdM) para la predicción de la anemia moderada-severa en fetos sometidos a transfusión y no sometidos. MÉTODOS: Se realizó una búsqueda sistemática para identificar estudios observacionales relevantes reportados en el período 2008-2018 que evaluaron el rendimiento de la MCA-PSV, utilizando un umbral de 1,5MdM para la predicción de la anemia fetal. El diagnóstico de la anemia fetal mediante la toma de muestras de sangre fue el estándar de referencia. Se utilizaron modelos de efectos aleatorios para la elaboración de una curva jerárquica resumen de las características operativas del receptor (hSROC, por sus siglas en inglés). Se realizaron análisis de subgrupos y metarregresión, según el número de transfusiones intrauterinas previas. RESULTADOS: En el metaanálisis se incluyeron doce estudios y 696 fetos. El área bajo la curva (ABC) hSROC para la anemia moderada-severa fue del 83%. La sensibilidad y especificidad agrupadas (IC 95%) fueron del 79% (70-86%) y 73% (62-82%), respectivamente, y los cocientes de verosimilitud positivos y negativos fueron 2,94 (IC 95%: 2,13-4,00) y 0,272 (IC 95%: 0,188-0,371). Cuando solo se consideraron los fetos no sometidos a transfusión, la predicción mejoró, pues se logró un ABC del 87%, una sensibilidad del 86% (IC 95%: 75-93%) y una especificidad del 71% (IC 95%: 49-87%). Se observó una disminución en la sensibilidad de la predicción de la anemia moderada-severa mediante la MCA-PSV ≥1.5MdM (estimación, -5,5% (IC 95%: -10,7 a -0,3%), P=0,039) en función del aumento del número de transfusiones previas. CONCLUSIONES: El uso de la MCA-PSV ≥1.5MdM para la predicción de la anemia moderada-severa en fetos no sometidos a transfusión muestra una precisión moderada (86% de sensibilidad y 71% de especificidad), que disminuye con el aumento del número de transfusiones intrauterinas.

A critical assessment of case reports describing absent uterus in subjects with oestrogen deficiency.

OBJECTIVE: The dual diagnosis of hypoplastic uterus in association with ovarian dysgenesis is regularly reported but the pathogenesis of the association is unclear. The uterus, however, may be invisible to all imaging modalities without at least six months of exogenous oestrogen exposure in complete ovarian failure. We assessed all available case reports in this category to estimate whether the apparent association between primary ovarian insufficiency or Turner syndrome and Mullerian agenesis can be largely accounted for by oestrogen deficiency. DESIGN: A literature review of all cases in which an association between ovarian insufficiency or Turner syndrome and hypoplastic uterus has been reported. PATIENTS: PubMed was searched for all case reports associated with relevant key terms. In total, 22 publications with a total of 25 patients were identified and reviewed; 14 subjects had the normal female karyotype (46,XX), and 11 subjects had Turner Syndrome. MEASUREMENTS: Proportion of subjects who had been exposed to adequate oestrogen prior to the absent uterine diagnosis. RESULTS: A diagnosis of absent uterus was made prior to exposure to exogenous oestrogen in 22/25 (88%) of subjects with primary hypogonadism including 14/14 females with normal karyotype and 8/11 females with Turner syndrome. CONCLUSIONS: Oestrogen deficiency is a possible explanation for most subjects being reported as having Mullerian agenesis in association with Turner syndrome or primary ovarian insufficiency. In the presence of oestrogen deficiency, no conclusion can be made about the status of the uterus until adequate exposure to exogenous oestrogen has been completed and we suggest reassessment of the uterus when full adult dose has been reached towards the end of induction of puberty.

Applicability of Magnetic Resonance Imaging in the Assessment of Fetal Urinary Tract Malformations.

OBJECTIVE: To assess the applicability of magnetic resonance imaging (MRI) to complement ultrasound in the diagnosis of fetal urinary tract anomalies. METHODS: This was a retrospective cohort study that included 41 women between 19 weeks and 37 weeks and 6 days of gestation carrying fetuses with malformations of the urinary tract which were initially diagnosed by ultrasound and then referred for MRI. In all cases, the diagnosis was confirmed after birth either through imaging or autopsy. A surface coil was positioned over the abdomen and T2-weighted sequences were obtained in the axial, coronal, and sagittal planes; T1 in at least one plane; and three-dimensional (3-D) TRUFI in fetuses with dilatation of the urinary tract. RESULTS: Mean gestational age at the time of MRI examination was 28.21 weeks. The rapid T2 sequences allowed all the anomalies of the fetal urinary tract to be assessed, whereas 3-D TRUFI sequencing proved very useful in evaluating anomalies involving dilatation of the urinary tract. The signs of pulmonary hypoplasia characterized by hypointense signal in the T2-weighted sequences were identified in 13 of the 41 fetuses. CONCLUSION: MRI confirmed and added information to the ultrasound regarding fetal urinary tract anomalies, as well as information related to the other associated malformations, their progress in the prenatal period, and possible postnatal prognosis.

Epidemiologic Assessment of Microtia in Over 23 Million Consecutive United States Births.

PURPOSE: Conflicting data exist regarding the incidence, demographics, and abnormalities associated with microtia. Using a large national cohort database, a comprehensive evaluation of microtia in the United States was performed. METHODS: The Kids' Inpatient Database was reviewed over a 15-year consecutive period. Information regarding patient demographics, comorbidities, postoperative complications, and hospital setting was collected. Statistical tests were analyzed using independent t tests and χ analysis. RESULTS: A total of 23,479,792 births over 15 consecutive years from 1997 to 2012 were included in the study. Microtia was identified in 1563 births. Incidence of microtia was higher in males (P<0.01) and patients of Asian or Hispanic race (P<0.01). Patients with microtia had significantly more congenital anomalies relative to the rest of the population (P<0.01). Specifically, cardiac and genitourinary anomalies were 51 and 19 times, respectively, more likely in microtia patients. Risk of associated congenital anomalies with microtia was highest in female and Black patients. 12.6% of all microtia patients had an associated craniofacial syndrome with a comparable postsurgical course to the nonsyndromic population. CONCLUSION: This study represents the largest national, longitudinal study of microtia allowing for demographic, socioeconomic, and comorbidity commentary. By quantifying relative risk of associated congenital anomalies, it can help inform the type and utility of screening modalities when evaluating microtia patients.

Challenges and Opportunities in Adolescent Gynecology Patients with Surgically-Treated Congenital and Acquired Anomalies: Transition of Care from Pediatric to Adult Surgery.

The transition from adolescence to young adulthood in patients with reproductive health care needs such as disorders of sexual development and congenital anomalies is a complex process that occurs over several years. The transition process for these patients is still poorly understood. The patients with disorders of sexual development and reproductive issues have specific and unique issues regarding timing of disclosure of diagnosis, genital examinations, gonadectomy, vaginal treatments, surgical procedures, hormone replacement therapy, use of long-term medication, and potential cancer screening. The purpose of this review is to briefly describe complex genital malformations and their associated anomalies with long-term concerns and then provide an overview of what has been published at this time regarding the transition of care to provide some guidance for providers who care for those patients.

Assessment of Congenital Vascular and Organ Anomalies in Subjects With Thalidomide Embryopathy Using Non-Contrast Magnetic Resonance Angiography.

BACKGROUND: To determine the type and frequency of vascular and organ malformations in adults with thalidomide embryopathy (TE) using non-contrast magnetic resonance angiography (MRA) and to assess the effect of the observed malformations on renal function. Methods and Results: The institutional ethics committee approved this prospective study and written informed consent was given by all 78 subjects (50 females) with TE (mean age: 55±1.1 years), who were examined by non-contrast MRA at 3T. ECG-triggered balanced turbo field echo images of the chest, abdomen and pelvis were obtained in coronal and sagittal orientations. Two observers assessed the frequency of vascular and organ malformations. Serum creatinine and estimated glomerular filtration rate (eGFR) were obtained to assess renal function. In 58 subjects, 99 vascular anomalies were observed, including 68 arterial (69%) and 31 venous anomalies (31%); 15 patients had 16 abdominal organ malformations including 12 kidney anomalies and 4 cases of gallbladder agenesis. Most vascular anomalies affected the renal vessels (n=66, 67%) or supraaortic arteries (n=28, 28%). Serum creatinine and eGFR revealed normal renal function in all subjects. CONCLUSIONS: Vascular and organ anomalies occurred in a high number of subjects with TE without evidence of renal dysfunction. Information about the presence of malformations may be important for future surgical interventions in subjects with TE.

The Mullerian Black Box: Predicting and defining Mullerian anatomy in patients with cloacal abnormalities and the need for longitudinal assessment.

INTRODUCTION: Most patients with a cloacal malformation have a Mullerian anomaly. We sought to examine our patients with a cloacal malformation to determine the proportion of them we felt we knew their Mullerian anatomy and which proportion we felt would most benefit from longitudinal assessment to define their anatomy, reproductive potential, and risk of outflow tract obstruction after puberty. We also compared the preoperative assessment of reproductive anatomy (pelvic ultrasound, MRI, cloacagram, and vaginoscopy) and intra-operative abdominal findings (at the time of primary cloacal reconstruction or subsequent abdominal procedures) to see how these correlated with and which preoperative assessment tool was most predictive of intraoperative anatomy. We also sought to confirm what we expected to be a lack of ovarian pathology. METHODS: A single site retrospective chart review was performed on all patients with a cloacal anomaly seen between May 2014 and September 2017. Preoperative assessment (pelvic ultrasound, MRI, cloaca gram, and vaginoscopy) and operative reports (both primary reconstruction and later abdominal procedures) were reviewed to ascertain Mullerian and ovarian anatomy. RESULTS: 30 of 36 (83%) of patients had defined Mullerian anatomy after preoperative assessment (pelvic ultrasound, MRI, cloacagram, and vaginoscopy) with or without the addition intraoperative assessment of Mullerian structures obtained during laparoscopy or laparotomy. 19/30 (63%) had duplication of their Mullerian structures. 25/36 (69%) had intraoperative assessment of Mullerian anatomy during laparoscopy or laparotomy. In this group, preoperative assessment with pelvic ultrasound correlated in 4/8 patients (50%), MRI correlated in 3/4 patients (75%), cloacagram in 10/15 patients (67%), and vaginoscopy in 23/25 patients (92%). 14/36 (39%) patients were found to require longitudinal assessment to define anatomy, reproductive potential or risk of outflow tract obstruction after puberty. Patients with ovarian findings described at the time of laparoscopy or laparotomy had no evidence of ovarian pathology. CONCLUSIONS: The majority of patients with cloaca in our series (83%) had their Mullerian anatomy defined by either preoperative assessment and/or findings at the time of laparoscopy or laparotomy. Duplication of the vagina and uterus was the most commonly described Mullerian anatomy (63%) in our series. Vaginoscopy appears to be superior to pelvic ultrasound, MRI, and cloacagram in predicting Mullerian anatomy. Fourteen of our 36 (39%) patients will require longitudinal assessment follow for reproductive potential and/or risk of outflow tract obstruction after puberty as their Mullerian anatomy is not known. There was no evidence of ovarian pathology in any cloaca patient. While we felt as though we could define Mullerian anatomy in most of our patients, any opportunity for intraoperative assessment of Mullerian anatomy should be utilized and therefore teams who are involved in the management of these patients must have a systematic and collaborative method established to ensure that Mullerian structures are thoroughly evaluated intra-operatively and documented in a standardized fashion. TYPE OF STUDY: Retrospective Chart review. LEVEL OF EVIDENCE: III.

PATIENT-SPECIFIC DOSIMETRY FOR PEDIATRIC IMAGING OF 99mTc-DIMERCAPTOSUCCINIC ACID WITH GATE MONTE CARLO CODE.

In this study, radiation absorbed dose of 99mTc-dimercaptosuccinic acid (DMSA) in critical organs was calculated using Monte Carlo simulation. Ten child patients with genitourinary abnormalities were imaged using a series of planar, SPECT and MRI, after injection with 99mTc-DMSA. Patient-specific organ segmentation was performed on MRI and used as input in GATE. Organs with substantial uptake included kidneys, bladder and liver. The mean organ absorbed dose coefficients (mGy/MBq) were 0.063, 0.058, 0.018, 0.016, 0.013 and 0.010 for the right kidney, left kidney, bones, urinary bladder wall, liver and gonads, respectively. The absorbed dose coefficients in the remainder of the body was 0.012 mGy/MBq. The authors implemented an image-based Monte Carlo method for patient-specific 3D absorbed dose calculation. This study also demonstrates the possibility to obtain patient-specific attenuation map from MRI to be used for the simulations of radiation transport and energy deposition in phantom using Monte Carlo methods.

Fundus Thickness Assessment by 3D Transvaginal Ultrasound Allows Metrics-Based Diagnosis and Treatment of Congenital Uterine Anomalies.

Purpose The development of a metric fundus assessment and definition of the uterine fundus thickness (FTH) for supporting objective diagnosis and treatment of congenital uterine anomalies (CUAs). Materials and Methods A) In a prospective cohort study, FTH was systematically assessed by 3 D transvaginal ultrasound (TVS) in patients, who presented a normal uterus. B) The mean normal FTH recorded was applied to uteri with fundal protrusions and septations, and compared to two CUA classification systems (ASF and ESHRE/ESGE). C) FTH was studied for preoperative metric planning and evaluation of the postoperative outcome. Results A) From August 2013 to January 2014, FTH was assessed in 100 normal uteri. An FTH of 12.02 +/- 2.03 mm (mean +/- SD) was recorded. B) Evaluating the 8 detected abnormal uteri, excellent correlation with the new ESHRE/ESGE classification system was observed, taking an FTH of 12 mm as the overall normal FTH equivalent to the individual normal wall thickness (WTH) stipulated in the classification and an FTH of 18 mm as the WTH + 50 %, serving as a cut-off value of (abnormal) septation. C) Metric fundus assessment was initially applied before and after septum resection and the following pregnancy could be monitored. Conclusion An FTH measurement in 3 D TVS paves the way for metric fundus diagnostics. This objectively distinguishes normal from abnormal fundi. It also has the potential to support accurate septum resection planning and objective postoperative evaluation. The value of the fundus assessment and metrics proposed in this study is not known. It should be investigated in prospective randomized controlled trials.

Continence in the cloacal exstrophy patient: What does it cost?

BACKGROUND: Surgical advancements have made cloacal exstrophy (CE) a survivable condition, though management remains complex. Urologic, orthopedic, colorectal and gynecologic interventions are not standardized, and the cost of this care is high. While the importance of a successful primary closure in terms of outcomes is known, the economic consequences of failure remain uncharacterized. METHODS: A prospectively maintained institutional database of epispadias-exstrophy complex patients was reviewed for continent CE patients. Hospital charges for all inpatient admissions prior to achieving urinary continence were inflation-adjusted to year 2013 values using Consumer Price Index for medical care published by the United States Bureau of Labor Statistics. Records for which charge data were incomplete were completed by using single mean imputation, also inflation-adjusted. Descriptive data are presented as mean±standard deviation (SD). RESULTS: Of 102 CE patients, 35 had available hospital charge data: 15 who underwent successful primary closure at the authors' institution and 20 who presented after previously failed primary closures at referring institutions. The mean±SD hospital charges for primary closure in the success group were $136,201±$48,920. These patients then underwent subsequent additional surgeries that accrued charges of $59,549±$25,189 in order to achieve continence. Overall, successful primary closures accumulated hospital charges of $200,366±$40,071. In comparison, patients referred after prior failure required significantly more hospital admissions and additional charges of $207,674±$65,820 were required to achieve continence (p<0.001). Patients who failed primary closure are estimated to accumulate 70% more total health care charges compared to the group following successful primary closure. CONCLUSION: The cost of CE management until urinary continence is high, averaging more than $200,000 in inpatient hospital charges alone. Initial success is desirable from both an outcomes and economic perspective, as the cost of salvaging a failed primary closure at our institution is similar to the overall costs of a successful closure; this is in addition to the cost of any previous failed closures. Further studies will be required to determine the optimal timing of surgical management in terms of both patient outcomes and financial consequences.

Assessment of kidney function in children by enzymatic determination of 2- or 24-h creatinine clearance: comparison with inulin clearance.

BACKGROUND: Although renal inulin clearance (Cin) is the gold standard for evaluation of kidney function, it cannot be measured easily. Therefore, creatinine clearance (Ccr) is often used clinically to evaluate kidney function. Enzymatically measured Ccr was recently found to be much higher than Cin because of the tubular secretion of creatinine (Cr). This study compared three measures of renal clearance, inulin, 2-h Ccr, and 24-h Ccr, in children. METHODS: Kidney function was evaluated in 76 children (51 males and 25 females) aged 1 month to 18 years with chronic kidney disease (CKD) by three renal clearance methods at almost the same time. RESULTS: Correlations between each pair of three renal clearance measurements were determined. Approximate glomerular filtration rate (GFR) was equal to 62 % of 2-h Ccr or 76 % of 24-h Ccr. CONCLUSION: Cr secretion by renal tubules was approximately 50 % of the GFR. In this study, we indicate that the measurements of 2-h Ccr or 24-h Ccr do not show true GFR but we could infer approximate GFR from the values. The use of 2- or 24-h Ccr might contribute to the treatment of pediatric CKD patients.

A retrospective review of telehealth services for children referred to a paediatric nephrologist.

BACKGROUND: Telemedicine has emerged as an alternative mode of health care delivery over the last decade. To date, there is very limited published information in the field of telehealth and paediatric nephrology. The aim of this study was to review our experience with paediatric telenephrology in Queensland, Australia. METHODS: A retrospective audit of paediatric nephrology telehealth consultations to determine the nature of the telehealth activity, reasons for referral to telehealth, and to compare costs and potential savings of the telehealth service. RESULTS: During a ten-year period (2004 - 2013), 318 paediatric telenephrology consultations occurred for 168 patients (95 male) with the median age of 8 years (range 3 weeks to 24 years). Congenital anomalies of the kidney and urinary tract (30 %), followed by nephrotic syndrome (16 %), kidney transplant (12 %), and urinary tract infection (9 %) were the most common diagnoses. The estimated cost savings associated with telehealth were $31,837 in 2013 (average saving of $505 per consultation). CONCLUSIONS: Our study suggests that paediatric telenephrology is a viable and economic method for patient assessment and follow up. The benefits include improved access to paediatric nephrology services for patients and their families, educational opportunity for the regional medical teams, and a substantial cost saving for the health care system.

Optimizing value utilizing Toyota Kata methodology in a multidisciplinary clinic.

INTRODUCTION: Value in healthcare is measured in terms of patient outcomes achieved per dollar expended. Outcomes and cost must be measured at the patient level to optimize value. Multidisciplinary clinics have been shown to be effective in providing coordinated and comprehensive care with improved outcomes, yet tend to have higher cost than typical clinics. We sought to lower individual patient cost and optimize value in a pediatric multidisciplinary reconstructive pelvic medicine (RPM) clinic. MATERIALS AND METHODS: The RPM clinic is a multidisciplinary clinic that takes care of patients with anomalies of the pelvic organs. The specialties involved include Urology, General Surgery, Gynecology, and Gastroenterology/Motility. From May 2012 to November 2014 we performed time-driven activity-based costing (TDABC) analysis by measuring provider time for each step in the patient flow. Using observed time and the estimated hourly cost of each of the providers we calculated the final cost at the individual patient level, targeting clinic preparation. We utilized Toyota Kata methodology to enhance operational efficiency in an effort to optimize value. Variables measured included cost, time to perform a task, number of patients seen in clinic, percent value-added time (VAT) to patients (face to face time) and family experience scores (FES). RESULTS: At the beginning of the study period, clinic costs were $619 per patient. We reduced conference time from 6 min/patient to 1 min per patient, physician preparation time from 8 min to 6 min and increased Medical Assistant (MA) preparation time from 9.5 min to 20 min, achieving a cost reduction of 41% to $366 per patient. Continued improvements further reduced the MA preparation time to 14 min and the MD preparation time to 5 min with a further cost reduction to $194 (69%) (Figure). During this study period, we increased the number of appointments per clinic. We demonstrated sustained improvement in FES with regards to the families overall experience with their providers. Value added time was increased from 60% to 78% but this was not significant. CONCLUSION: Time-based cost analysis effectively measures individualized patient cost. We achieved a 69% reduction in clinic preparation costs. Despite this reduction in costs, we were able to maintain VAT and sustain improvements in family experience. In caring for complex patients, lean management methodology enables optimization of value in a multidisciplinary clinic.