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Countless papers have been published regarding the management and clinical outcome of vesicoureteral reflux (VUR), still no active treatment has been proven superior to another, regarding preserving renal function. When considering comparable treatment alternatives, qualitative research is needed to understand the parents' perspectives and preferences. This study aims to describe the parents' experiences of infant high-grade VUR (hVUR) regarding continuous antibiotic prophylaxis (CAP), surgical intervention (SI), urinary tract infection (UTI) and renal damage. We performed four randomized, semi-structured focus groups (FG) with 19 parents to 15 children (aged 1,5–6 years). All children had been diagnosed with hVUR at <8 months of age and treated with CAP (all groups) and SI (two groups). Discussions were recorded, transcribed and analysed to content. The sample size for the FGs was based on category saturation, which was confirmed through comparison analysis in multiple FGs. The FGs generated 2,897 parent-reported experiences, of which this study reports on 1,123, sorted into the abovementioned four themes and underlying categories. Negative experiences regarding CAP, such as stress regarding the daily intake and worries about long-term use and side effects, were abundant, whereas positive experiences were few. The experiences regarding SI were negatively affected by inadequate information and postoperative difficulties and positively by empathy, accurate information and adequate preparations. The increased risk of UTIs were described as a constant emotional stress causing restricted social activities, frequent visits to the hospital and challenges regarding urine-sampling. There was a common awareness of renal damage, but few experiences reflected any actual worry. The daily struggle with medications and monitoring for symptoms, concerns of future antibiotic resistance and a parental preference of SI have been documented in previous studies. FG methodology effectively collects data from several participants during the same occasion, the goal being to generate discussions that enable researchers to see the world from the participants' perspective. Since the management of infants with hVUR is still under debate, qualitative research can remind of valuable patient and parent perspectives. This study shows that CAP and the risk of UTI have non-negligible, everyday impact on family life, while renal damage seems of secondary importance. The concerns of surgical treatment are related to an isolated occasion, which can be optimized with proper care and improved preoperative preparations. Awareness of parents' experiences and preferences is helpful when managing children with hVUR. Summary table Summary table Themes and categories n = 1123 statements Parents' experiences of continuous antibiotic prophylaxis, CAP n=320 Emotional impact n = 140 (44%) Concerns about the effect of CAP n = 96 (30%) Influence of CAP intake n = 84 (26%) Parents' experiences of surgical intervention, SI n=182 Difficulties in surgical care n = 94 (52%) Qualities in surgical care n = 88 (48%) Parents' experiences of urinary tract infection (UTI) n=535 Family impact due to the child's UTI n = 273 (51%) Emergency care at suspected UTI of the child n = 131 (24%) Obstacles and facilitators of sampling and testing of urine n = 131 (24%) Parents' experiences of renal damage n=86 Risk of renal damage n = 86 (100%) [ABSTRACT FROM AUTHOR]

The management of Differences of Sex Development (DSD) has evolved considerably in recent years. The questioning of systematic early childhood treatment of DSD requires a better understanding of the outcomes of such treatments and long-term studies are therefore essential to better evaluate the prognosis of DSD. Unfortunately, limitations are numerous including the limited size of the series, the absence of standardized methodology, the evaluation of managements that no longer take place today and the absence of prospective and comparative studies. Despite these difficulties, the purpose of this paper is to present the current data on the long-term follow-up of patients with DSD from the urological, sexual and fertility points of view. Even if it remains difficult at present to establish precise recommendations, we recapitulate the most important points that should drive follow-up of these patients especially the constitution of a multidisciplinary team with a holistic approach, the organization of the transition between adolescence and adulthood, a particular attention to psychological care, a careful communication with the patients and his/her family and the use of standardized data collection systems. [ABSTRACT FROM AUTHOR]

Adrenalectomy is routinely performed via the minimally invasive approach. Safety of adrenalectomy using the robot-assisted technique has been widely demonstrated by several series, but the literature is scarce regarding the comparison of conventional laparoscopic versus robot-assisted approach. We decided to carry out a multicenter study to compare clinical and surgical outcomes between laparoscopic and robotic adrenalectomy. This is a retrospective case-control study, including data from centers affiliated to the Surgical Registry EUROCRINE. Patients undergoing laparoscopic surgery for adrenal tumors and registered between 2015 and 2018 were included. Robot-assisted versus laparoscopic adrenalectomy was compared. All comparisons were carried out in terms of complication rate, conversion rate and duration of stay. A total of 1,005 patients from 46 clinics underwent robotic or conventional laparoscopic adrenalectomy. Median age was 55 (interquartile range: 45−65) years. Robotic adrenalectomy was performed in 189 (18.8%) patients. According to Clavien-Dindo classification, complication rate was lower in the robotic surgery group (1.6% vs 16.5%, P <.001). Laparoscopic surgery and active hormonal status were significantly correlated with complications, both in univariate and multivariate analysis. There was no significant difference between laparoscopic and robotic surgery groups, in terms of conversion rate (2.1% vs 0.5%, respectively, P =.147). Duration of stay was shorter in the robotic adrenalectomy group (82.1% vs 28.8%, P <.001). Analysis of the EUROCRINE database supports that robotic adrenalectomy resulted in a lower complication rate and shorter duration of stay, compared with laparoscopic adrenalectomy. Granular data to support this is warranted. [ABSTRACT FROM AUTHOR]

Permanent hypoparathyroidism is common after thyroidectomy. The present study evaluated the risk for morbidity in patients operated with total thyroidectomy with and without permanent hypoparathyroidism. Data was retrieved from the Scandinavian Quality Register for Thyroid, Parathyroid, and Adrenal Surgery and cross-linked with the Swedish National Prescription Registry for Pharmaceuticals, the National Data Inpatient Registry, and Causes of Death Registry. Patients with benign thyroid disease were included. Permanent hypoparathyroidism was defined as treatment with active vitamin D for more than 6 months after thyroidectomy. Analyzed morbidity was evaluated by multivariable Cox's regression analysis and presented as hazard ratio and 95% confidence interval. There were 4,828 patients. The mean (standard deviation) follow-up was 4.5 (2.4) years. Some 239 (5.0 %) patients were medicated for permanent hypoparathyroidism. Patients with permanent hypoparathyroidism had an increased risk for renal insufficiency, hazard ratio 4.88 (2.00–11.95), and an increased risk for any malignancy, hazard ratio 2.15 (1.08–4.27). Patients with permanent hypoparathyroidism and known cardiovascular disease at the time of thyroidectomy had an increased risk for cardiovascular events during follow-up, hazard ratio 1.88 (1.02–3.47). Patients with permanent hypoparathyroidism after total thyroidectomy have an increased risk of long-term morbidity. These results are a cause of great concern. [ABSTRACT FROM AUTHOR]

Summary Introduction High-grade vesicoureteral reflux (VUR) in children is associated with recurrent urinary tract infection (UTI) and renal damage. Breakthrough UTI despite continuous antibiotic prophylaxis (CAP) during the first years of life is a matter of concern and evokes early intervention. We investigated whether early endoscopic treatment (ET) of VUR grade 4–5 can reduce the risk of UTI recurrence and renal scarring. Materials and methods This prospective, randomized, controlled, multicentre, 1-year follow-up trial comprised 77 infants, <8 months of age with VUR grade 4–5 (Table) randomized to CAP ( n = 39) or ET (with prophylaxis until resolution) ( n = 38). Voiding cystourethrogram, ultrasound, renal scintigraphy, and free voiding observation were performed at study entry and after 1 year. Parenchymal defects were seen in 67 (87%) children at entry, 39 (34 boys, 5 girls) of them characterized as generalized. At follow-up, renal deterioration (new scars or progress in old damaged area) and symptomatic UTIs were reported. Results There were 27 recurrent febrile UTIs in 6 (16%) children in the ET group and in 10 (26%) in the CAP group ( p = 0.43), in eight (36%) girls and eight (15%) boys ( p = 0.039). Successful VUR outcome (VUR 0–2) was seen in 22 (59%) in the ET and eight (21%) in the CAP group ( p = 0.0014). Multiple recurrences were only seen in patients with persistent dilating reflux at follow-up ( p = 0.019). Deterioration on scintigraphy was seen in eight children (9 kidneys) with no difference between treatment groups ( p = 0.48) or sex ( p = 0.17). Renal deterioration was associated with high bladder capacity (BC) and large residual volume (PVR) at 1 year ( p = 0.0092 and p = 0.041). Six of the eight children with renal deterioration had a recurrent UTI ( p = 0.0032). Seven of nine renal units with deterioration were seen in children with persistent VUR 3–5 at follow-up. Univariable logistic regression identified female sex and high PVR as positive predictors for recurrent UTI ( p = 0.039 and 0.034) and high PVR tended to predict renal deterioration ( p = 0.053). Discussion No differences between the treatment groups regarding recurrent UTI and renal deterioration could be found. Increased PVR and female sex were positive predictors for UTI recurrences. VUR grade at follow-up was correlated to UTI recurrence and renal deterioration. Conclusion This study did not show any difference between ET and CAP in reducing the risk of UTI recurrence or renal deterioration. The rate of VUR resolution was higher in the ET group and VUR grade at follow-up correlated with both UTI recurrence and renal deterioration. Table Baseline demographics and the primary outcome in the total study population and per treatment group. Table Variable Total ( n = 77) Endoscopic treatment ( n = 38) Prophylaxis ( n = 39) p Baseline Sex Girls 22 (29%) 12 (32%) 10 (26%) Boys 55 (71%) 26 (68%) 29 (74%) 0.75 Age at randomization (months) 6.7 (4.1–9.2) 6.8 (4.1–9.2) 6.7 (4.7–8.8) 0.65 Grade of VUR 4 30 (39%) 15 (40%) 15 (39%) 5 47 (61%) 23 (61%) 24 (62%) 1.00 Bilateral VUR 52 (68%) 25 (69%) 27 (69%) 0.94 Renal damage 67 (87%) 32 (84%) 35 (90%) 0.70 1-year follow-up Febrile UTI during study 16 (21%) 6 (16%) 10 (26%) 0.43 Renal deterioration 8 (11%) 3 (8%) 5 (14%) 0.74 Note . For categorical variables n (%) is presented. For continuous variables mean (range) is presented. [ABSTRACT FROM AUTHOR]

Summary Introduction High-grade vesicoureteral reflux (VUR) in infants is associated with congenital renal abnormalities, recurrent UTI, and bladder dysfunction. Endoscopic treatment (ET) is a well-established method in children with low to moderate reflux grades, but there is a lack of randomised controlled trials regarding the use of ET versus continuous antibiotic prophylaxis in infants with high-grade VUR. Objective This study aimed to determine whether high-grade VUR in infants can be treated with endoscopic injection and whether ET is superior to antibiotic prophylaxis in the treatment of VUR. Materials and methods This prospective, randomised, controlled, multicentre, 1-year follow-up trial comprised 77 infants (55 boys, 22 girls) <8 months of age with VUR grade 4–5 ( n = 30/ n = 47). Of the infants, 52 (68%) had bilateral VUR. Thirty-nine were randomised to antibiotic prophylaxis and 38 to ET (with prophylaxis until resolution). Voiding cystourethrogram, ultrasound, renal scintigraphy, and free voiding observation were performed at study entry and after 1 year to evaluate VUR grade, and renal and bladder function. Results VUR grade ≤2 was seen in 22 (59%) infants in the endoscopy group and eight (21%) in the prophylaxis group at follow-up ( p = 0.0014). The success rate in the endoscopy group was 100% in unilateral grade 4, falling to 31% in bilateral grade 5 ( p = 0.0094). Correspondingly, the results in the prophylaxis group were 40% in grade 4 down to 0% in bilateral grade 5 ( p = 0.037) ( Table ). Logistic regression analyses identified ET, VUR grade 4, unilaterality, and low residual urine at baseline as positive predictors of VUR down-grading to ≤2 (area under ROC curve 0.88). In four patients with reflux resolution after one injection, dilating reflux recurred at the 1-year follow-up. One patient had a UTI possibly related to ET. In our material four patients required re-implantation, of whom one was obstructive after injection. Discussion The opportunity to offer even small infants with high-grade VUR an alternative, minimally invasive treatment option is a great advance in paediatric urology. In this high-risk group, bilateral VUR grade 5 stands out with its poor bladder function and low chance of resolution. The recurrence rate of dilating VUR after successful ET is consistent with previous studies. The limitations are the relatively small number of patients and the short follow-up. Conclusion High-grade VUR in infants can be treated with injection therapy and the resolution rate is higher than that of prophylaxis treatment. The complication rate is low and VUR grade 4, unilaterality, and low residual urine are favourable for the resolution and down-grading of VUR. Table VUR-grade at 1-year follow-up and VUR ≤2 divided by VUR-grade and uni- or bilaterality at baseline trial. Variable Endoscopy group ( n = 38) Prophylaxis group ( n = 39) p -value Grade of VUR at 1-year follow-up VUR = 0 17 (46%) 6 (16%) VUR = 1 2 (5%) 0 (0%) VUR = 2 3 (8%) 2 (5%) VUR = 3 6 (16%) 7 (18%) VUR = 4 4 (11%) 13 (34%) VUR = 5 5 (14%) 10 (26%) 0.0007 VUR grade ≤2 vs >2 at 1-year follow-up VUR grade 0–2 22 (59%) 8 (21%) VUR grade 3–5 15 (41%) 30 (79%) 0.0014 Variable Endoscopy group Prophylaxis group VUR grade ≤2 at 1-year follow-up ( n = 22) VUR grade >2 at 1-year follow-up ( n = 15) p -value VUR grade ≤2 at 1-year follow-up ( n = 8) VUR grade >2 at 1-year follow-up ( n = 30) p -value VUR grade at Baseline Unilateral 4 7 (100%) 0 (0%) 2 (40%) 3 (60%) Bilateral 4 6 (75%) 2 (25%) 4 (40%) 6 (60%) Unilateral 5 4 (67%) 2 (33%) 2 (29%) 5 (71%) Bilateral 5 5 (31%) 11 (69%) 0.0094 0 (0%) 16 (100%) 0.037 [ABSTRACT FROM AUTHOR]

Summary Introduction It has been suggested that infants with high-grade vesicoureteral reflux (VUR) have lower urinary tract dysfunction (LUTD) that is characterised by large bladder capacity (BC) and increased post-void residual (PVR). However, most of these infants have normal or small BC in early infancy and develop large capacity during the first year of life. Objective This study aimed to see whether LUTD development during the infant years in children with high-grade VUR could be prevented by early reflux resolution. Materials and methods For early VUR intervention, endoscopic treatment (ET) was used in a randomised trial comprising 77 infants (55 boys) aged <8 months with VUR grade 4–5 (n = 30/n = 47); 39 were randomised to antibiotic prophylaxis and 38 to ET. Voiding cystourethrogram, free voiding observation (FVO) and renal scintigraphy were performed at baseline and after 1 year. Bladder capacity and PVR were obtained from FVO. LUTD was defined as a BC of ≥150% of expected and a PVR of ≥20 ml. Results There were no differences in bladder function variables seen between the treatment groups, despite significant differences in VUR resolution. Analysing bladder function related to VUR outcome (VUR grade ≤2 vs grade >2), independent of treatment, showed that VUR grade ≤2 was associated with a smaller BC at 1 year ( P = 0.050) (a tendency already seen at baseline) and a lower PVR at baseline ( P = 0.010). PVR increased from baseline to 1 year ( P = 0.037) in children with grade ≤2 VUR (Summary Table). The group with persistent bilateral grade 5 VUR at 1 year had more abnormal bladder variables compared with other study subjects, with a tendency of larger BC ( P = 0.057), higher PVR ( P = 0.0073) and more LUTD ( P = 0.029) at baseline and a larger BC at 1 year ( P = 0.016). In explanatory analyses, using logistic regression, a high PVR at baseline was identified as a predictor of VUR grade >2 ( P = 0.046), persistent bilateral grade 5 VUR ( P = 0.022), recurrent urinary tract infection ( P = 0.034), and only a tendency was seen regarding new renal damage ( P = 0.053). Conclusion There was no between-group difference seen in bladder function. In children with VUR resolution at follow-up, independent of treatment, BC decreased, whereas PVR increased. High PVR at baseline was a predictive factor for both non-resolution of high-grade VUR and recurrent urinary tract infection. The results suggest that LUTD cannot be prevented by early VUR resolution, but rather is an important prognostic factor for VUR outcome in both endoscopic and prophylactic treatment. Summary Table Bladder variables by grade ≤2 vs grade >2 VUR at 1-year follow-up. Variable Total
(n = 74) Within-group P -value Grade ≤2 VUR at 1 year 
(n = 29) Grade >2 VUR at 1 year 
(n = 45) Between-group P -value Bladder capacity (%) • Baseline 126 (49–466) 120 (49–283) 138 (65–466) 0.14 • 1 year 113 (3–296) 0.075 90 (31–194) 121 (30–296) 0.050 • Large (≥150%) baseline 28 (41%) 7 (27%) 21 (49%) 0.12 • Large (≥150%) 1 year 22 (33%) 7 (26%) 15 (39%) 0.43 Residual urine (ml) • Baseline 9 (0–83) 6 (0–55) 11 (1–83) 0.010 • 1 year 14 (0–108) 0.025 9 (0–84) 16 (0–108) 0.41 Lower urinary tract dysfunction • Baseline 17 (24%) 3 (12%) 14 (33%) 0.087 • 1-year follow-up 18 (27%) 6 (22%) 12 (31%) 0.63 Continuous variables: median (minimum−maximum), categorical variables: n (%). [ABSTRACT FROM AUTHOR]

Introduction: The external branch of the superior laryngeal nerve is important for the voice. However, it is unknown how thyroid surgeons manage this nerve during operations. We hypothesised that this management is related to the surgeon's age, gender, the surgeons' annual number of thyroid operations, i.e volume, and surgical specialisation.Methods: We sent a 17-item enquiry by e-mail to members of the Swedish Endocrine Surgical Society and the Swedish Association for Otorhinolaryngology--Head and Neck Surgery. The questions covered subjects such as age, gender, type of department and operation volume of the surgeon, and whether and how the surgeon would try to preserve and/or identify the EBSLN during an operation. Surgeons were asked to rate the importance of the nerve, and of their use of laryngoscopy and/or voice recording.Results: There were 85 respondents. Ninety per cent of them would always or often try to preserve the nerve, but only half would always or often try to identify it. Endocrine surgeons more often stated that they would preserve the nerve; they rated its importance more highly and they more often mentioned the use of neuromonitoring to identify the nerve, than head and neck surgeons. Head and neck surgeons on the other hand were more likely to perform laryngoscopy. Endocrine surgeons more often performed >50 thyroid operations per year than head and neck surgeons.Conclusions: Management of the external branch of the superior laryngeal nerve differed according to surgeon specialisation, which in turn was associated with surgical volume. [ABSTRACT FROM AUTHOR]

Introduction The external branch of the superior laryngeal nerve is important for the voice. However, it is unknown how thyroid surgeons manage this nerve during operations. We hypothesised that this management is related to the surgeon's age, gender, the surgeons' annual number of thyroid operations, i.e volume, and surgical specialisation. Methods We sent a 17-item enquiry by e-mail to members of the Swedish Endocrine Surgical Society and the Swedish Association for Otorhinolaryngology – Head and Neck Surgery. The questions covered subjects such as age, gender, type of department and operation volume of the surgeon, and whether and how the surgeon would try to preserve and/or identify the EBSLN during an operation. Surgeons were asked to rate the importance of the nerve, and of their use of laryngoscopy and/or voice recording. Results There were 85 respondents. Ninety per cent of them would always or often try to preserve the nerve, but only half would always or often try to identify it. Endocrine surgeons more often stated that they would preserve the nerve; they rated its importance more highly and they more often mentioned the use of neuromonitoring to identify the nerve, than head and neck surgeons. Head and neck surgeons on the other hand were more likely to perform laryngoscopy. Endocrine surgeons more often performed >50 thyroid operations per year than head and neck surgeons. Conclusions Management of the external branch of the superior laryngeal nerve differed according to surgeon specialisation, which in turn was associated with surgical volume. [ABSTRACT FROM AUTHOR]

Purpose We present data on long-term functional and cosmetic results after hypospadias surgery. Materials and Methods Males older than 18 years with hypospadias treated in Sweden were asked to participate in the study, as well as age matched controls and circumcised men. All participants answered questionnaires, and a subgroup was examined during an outpatient visit. Relationships with outcome were analyzed using analysis of variance and regression analysis. Results A total of 167 patients with a mean age of 34 years and 169 controls with a mean age of 33 years answered the questionnaire. Of the patients 63% had distal, 24% mid and 13% proximal hypospadias. A total of 46 patients and 49 controls presented for physical examination. Patients were significantly less satisfied with the penile cosmetic outcome regarding all parameters of the Penile Perception Score. There was a difference in penile length between patients and controls (mean 9.7 vs 11.6 cm, p <0.001). More patients than controls reported voiding dysfunction symptoms (p = 0.003). Patients had a lower maximum urinary flow rate than controls (p = 0.001). These differences were most prominent between patients with proximal hypospadias and controls. Conclusions Men operated on for hypospadias were less satisfied with the cosmetic result than controls, and had a shorter penile length. Patients presented with more symptoms of voiding dysfunction and displayed a lower maximum urinary flow rate. Patients with proximal hypospadias were more affected than those with milder hypospadias. Our results indicate that patients with hypospadias can be subgrouped and that those with severe phenotypes should be followed more closely during childhood as well as later in adulthood. [ABSTRACT FROM AUTHOR]

Purpose: We studied the incidence of hypospadias in Sweden during a 40-year period to determine if changes were associated with known risk factors. Materials and Methods: We analyzed prospective data from nationwide health care and demographic registers collected for all males (1,948,591 total) born in Sweden between 1973 and 2009. The incidence of hypospadias per 1,000 live-born boys was calculated as number of cases divided by total number of births yearly. The association between hypospadias and risk factors was estimated using logistic regression, expressed as odds ratios. Results: The nationwide incidence of boys diagnosed with hypospadias was approximately 4.5 per 1,000 live-born boys until 1990, increasing to 8 per 1,000 boys during the following decade. Mild and severe phenotypes comprised the increase. Boys born small for gestational age (OR 4.34), as a twin (OR 1.8), as a result of in vitro fertilization (OR 1.15), or with parents from Asia (OR 1.45) or continental Europe (OR 1.41) were at increased risk for hypospadias. Multivariate analyses revealed that changes in risk factors did not explain the increased incidence. However, a systematic change in the classification of the diagnosis in registers could not be ruled out. Conclusions: This nationwide study demonstrates an increased incidence of hypospadias diagnoses in Sweden from 1990 to 1999 that is not attributable to previously known risk factors. The increase includes mild and severe phenotypes, suggesting that shifts in the diagnostic criteria are not the underlying cause. [ABSTRACT FROM AUTHOR]

Objective: To determine the relationship between repeat courses of antenatal corticosteroids (ACS) and risk factors for cardiovascular disease in adolescents and young adults. Study design: We assessed body mass index, blood pressure, arterial stiffness, blood lipids, and insulin resistance (IR) in a Swedish population-based cohort (n = 100) at a median age of 18 (range 14-26) years. Fifty-eight subjects (36 males) had been exposed to 2-9 weekly courses of antenatal betamethasone and 42 (23 males) were unexposed subjects matched for age, sex, and gestational age (GA). Results: There were no significant differences between the groups regarding body mass index, systolic or diastolic blood pressures, arterial stiffness measured by augmentation index, blood lipids, IR, or morning cortisol levels either in simple regression or in multivariable models. However, more subjects with elevated augmentation index had been exposed to repeat courses of ACS (n = 7) compared with unexposed subjects (n =1, P = .06), and glucose, insulin, and IR correlated inversely to GA at start of ACS (P < .01). Conclusions: Repeat courses of ACS did not correlate to adverse cardiovascular risk profile in adolescence and young adulthood, but long-standing effects on the arterial tree and glucose metabolism, the latter dependent on GA at ACS exposure, cannot be excluded. These observations have clinical implications for the ongoing discussion on short-term benefits and long-term safety of repeat ACS treatment. [Copyright &y& Elsevier]

Abstract: Existing outcomes for DSD individuals are inadequate because reports are based upon information collected retrospectively. This paper is presented to review existing data emphasizing information needed to lead to better future care, is based on presentations and discussions at a multi-disciplinary meeting on DSD held in Annecy in 2012, and is not intended to define the present status of management of each of the various DSD diagnoses. Rather it is intended to provide information needed to do studies regarding outcome data from the treatment of children with DSD by providing a summary of recommendations of ‘patient-centered’ topics that need investigation. The hope is that by being concerned with what is not known, new protocols will be developed for improving both early management and transition to adult life. [Copyright &y& Elsevier]

Abstract: This paper is a review of some of the recent publications regarding outcome of DSD patients, with an emphasis upon surgical and sexual outcomes. Currently available outcome studies of patients with DSDs have limitations because of multiple factors, including lack of representative patient sampling, and lack of adequate information concerning both medical and surgical care, and psychological, social and family support. The most frequent reports involve females with 21-α-hydroxylase deficiency congenital adrenal hyperplasia (CAH). This most common form of DSD, if one excludes hypospadias and cryptorchidism, is an excellent example of a form of DSD in which all aspects of outcome, regarding surgery, sexual functionality and sensitivity, psychological input and endocrine hormonal therapy, carry a major role. The goals of therapy include a surgical outcome with a good cosmetic appearance and functionality with potential for sexual intercourse with sufficient sensitivity for satisfactory responsiveness. Endocrine replacement therapy should provide a normal adrenal hormonal milieu, while sex steroid therapy may be indicated. Psychological care should be provided from birth with gradual transition primarily to the patient, including basic counseling with full disclosure, although adjustment depends upon the patient''s personality and parents'' abilities and acceptance. Among forms of DSD involving gonadal insufficiency, hormonal replacement therapy should provide physiologic levels. Among females, estrogen therapy enhances healing after feminizing surgery and is required from puberty throughout adult life to maintain femininity, sexual organs and bone health, and enhance gender and sexuality. Among males, appropriate testosterone therapy maintains stamina, muscle tone, bone health, libido, sexual potency and general well-being, while benefit for healing after genital surgery is unclear. Further, outcome is clearly related to predominant cultural factors. Outcome studies should include evaluation of all of these factors. [Copyright &y& Elsevier]

Abstract: This study examines the effect of glucose and insulin on the plasma elimination rate and oxidation of a fat emulsion by using indirect calorimetry in conjunction with lipid and insulin-glucose clamp techniques. Ten healthy subjects were studied on 2 occasions in a randomized, open, crossover study. On one occasion, a hypertriglyceridemic (HTG) clamp was administrated alone; and, on the other, HTG and insulin-glucose (IG) clamps were administrated simultaneously. During HTG clamps, serum triglyceride (TG) concentration was maintained at 4 mmol × L−1. During the IG clamp, insulin was administered at a rate of 20 mU × m−2 × min−1; and the glucose level was maintained at 7 mmol × L−1. Continuous indirect calorimetry was carried out throughout the study period. The infusion rate required to maintain stable serum TG concentrations did not differ between the 2 clamps. Mean free fatty acid concentration was lower during the HTG/IG than during the HTG clamp (0.40 ± 0.04 vs 0.82 ± 0.07 mmol × L−1; P < .001). However, the increases in β-OH-butyrate levels were significantly lower in the HTG/IG compared with the HTG clamp (0.09 ± 0.04 vs 0.55 ± 0.09 mmol × L−1; P < .001). Energy expenditure and the respiratory quotient were significantly higher at steady state in the HTG/IG than in the HTG clamp: 1.47 ± 0.06 vs 1.34 ± 0.04 kcal × min−1 (P < .01) and 0.85 ± 0.01 vs 0.79 ± 0.01 (P < .01), respectively. Insulin and glucose did not significantly change plasma TG disposal rate (P = .0987) or total lipid oxidation (P = .3204) in this metabolic situation with an abundant supply of both carbohydrates and lipids. β-OH-butyrate increased during both clamps, indicating an ongoing hepatic fatty oxidation despite the administration of glucose/insulin. [Copyright &y& Elsevier]

Summary: Infants born with ambiguous genitalia represent a complex clinical challenge. A systematic clinical investigation aims at determining the hormone production and which anatomical structures are present in order to understand at what level the sex differentiation has been affected; chromosomal, gonadal or hormonal synthesis and action levels. The increased genetic knowledge in the field has opened up new diagnostic possibilities. Sex development requires the balanced and sequential activation of transcription factors, signaling molecules and hormones. It has recently been shown that not only testis but also normal ovarian development is an active process. Genes involved in gonadal disorders of sex development often act in a gene dosage-dependent manner, with different effects in XY or XX embryos. The management of patients with disorders of sex development, including decisions about sex of rearing, must be carried out by a specialized multidisciplinary team and include an extended genetic investigation as well as psychological considerations. [ABSTRACT FROM AUTHOR]

Abstract: Background: Because of its high accuracy, dual-energy X-ray absorptiometry (DXA) has become one of the most frequently used methods for estimating human body composition. One limiting factor concerning measuring obese people with the DXA technique is the size of the scanning area. Objective: To explore the possibility of estimating whole body composition from half-body scans before and after weight reduction, and compare the results with densitometry measurements. Design: Intervention study of 15 obese adults (age 47.2±13.4; BMI 35.9±3.1) who were measured with full- and half-body DXA scans before and after a 7-week weight loss program. On both occasions, body composition was also assessed with air-displacement plethysmography (ADP). Results: The mean weight loss at follow-up was 14.9±4.1kg (5.0kg/m2), corresponding to a 14% decrease in body weight. When comparing the results from full- and half-body DXA, between 96% and 98% of the variance was explained. At baseline, %Body Fat (%BF) did not differ significantly between full and half-body measurements (0.6, −0.1−1.3), but the half-body method overestimated it by 1.0% (0.2–1.8) at follow-up. On the contrary, the difference between DXA and ADP in the assessment of %BF was both significant and of large magnitude (5.2; 2.4–8.0) at baseline, while non-significant and near zero (0.4; −1.3–2.2) at follow-up when the subjects had lost a significant amount of weight. Conclusion: The results obtained from half-body DXA scans can accurately predict whole body composition, as measured by full-body DXA, before and after significant weight reduction, in obese patients who barely fit into the scanning area. However, increasing discordance between DXA and ADP with increasing adiposity was seen, indicating that the measurements might not be as reliable on extreme obese subjects as on normal and overweight ditto. [Copyright &y& Elsevier]

Abstract: In a randomized crossover study, plasma kinetics of 2 different types of fat emulsions were studied in 8 healthy volunteers by using a hypertriglyceridemic clamp technique. The method involves the stabilization of serum triglyceride (TG) concentration during 180 minutes at a predetermined level (4 mmol/L) by adjustment of TG infusion rate by repeated online measurements of serum TG concentration. The fat emulsions under study were a long-chain fatty acid triglyceride (LCT) emulsion (Intralipid 20%, Fresenius Kabi, Sweden) and a structured triglyceride (STG) emulsion (Structolipid 20%, Fresenius Kabi) where medium- and long-chain fatty acids have been interesterified within a TG molecule. The hypertriglyceridemic clamp was found to have acceptable reproducibility when tested in 3 healthy individuals on 2 different occasions, as similar steady-state TG levels were obtained by infusing similar amounts of fat. The average (±SEM) TG concentration during the 180-minute clamp was similar for STGs and LCTs (4.0 ± 0.1 vs 3.9 ± 0.1 mmol/L; not significant), but the amount of fat that had to be infused was significantly higher during STG than during LCT clamping (0.31 ± 0.04 vs 0.21 ± 0.02 g TG per minute; P < .05). Higher serum levels of free fatty acids (1.80 ± 0.13 vs 0.96 ± 0.09 mmol/L; P < .05), free glycerol (1.30 ± 0.07 vs 0.76 ± 0.08 mmol/L; P < .001), and β-OH butyrate (1.61 ± 0.44 vs 1.17 ± 0.23 mmol/L; not significant) were obtained at the end of the clamp during infusion of STGs compared with LCTs. During infusion of STGs the medium-chain fatty acids octanoic (C:8) and decanoic acid (C:10) constituted approximately half of circulating fatty acids that correspond to the compositional ratio of the emulsion. Plasma lipoprotein lipase (LPL) concentration was higher during STG than during LCT clamping (6.06 ± 0.62 vs 3.15 ± 0.40 mU/mL; P < .05), and there was a positive correlation between the mean LPL concentration and the amount of infused TG during the steady-state period (r = 0.58; P < .05). In conclusion, the hypertriglyceridemic clamp method was found to give reproducible results and could be considered for comparison of metabolic clearance properties of different types of fat emulsions. The capacity of healthy subjects to eliminate STGs from blood was greater than for LCTs. An increased LPL activity induced by the higher TG infusion rate may have contributed to the increased metabolic clearance of STGs. [Copyright &y& Elsevier]