The age-corrected SD scores for testicular volume will aid in the diagnosis and follow-up of abnormalities in the timing and progression of male puberty and in research evaluations. The SD scores can be compared with pubic hair SD scores to identify discrepancies between cell functions that result in relative microorchidism or macroorchidism.

Summary This study analyzed the mortality and causes of death in sickle cell disease patients in the Netherlands, to provide a baseline for monitoring the effect of the recently introduced neonatal screening programme and to indicate areas of improvement in the care for these patients. All children (<18 years) diagnosed with sickle cell disease in a tertiary hospital from 1985 to 2007 were included. Vital status was determined up to March 2008. A total of 298 children were included: 189 (63%) patients had HbSS, 17 (6%) HbSβ0 thalassaemia, 72 (24%) HbSC and 20 (7%) HbSβ+ thalassaemia. Twelve patients (4%) died during a total follow‐up of 3896 patient years. All known deaths were sickle cell disease‐related. Meningitis/sepsis (n = 4; 33%), stroke (n = 3; 25%) and death during a visit to the country of origin (n = 3; 25%) were the most common causes of death. The overall mortality rate was 0·27 deaths/100 patient years [95% confidence interval (CI): 0·15–0·43]. The estimated survival at the age of 18 years was 97·3% (95% CI: 95–99%). This report confirms that the burden of mortality in sickle cell disease is increasingly shifting to adults. It is recommended that compliance to antibiotic prophylaxis, thorough counselling and support for patients travelling abroad and specialized peri‐operative care should receive continuous attention.

The long-term volumes at diagnosis of acquired undescended testes after orchiopexy were significantly less than the normative values at all ages. In unilateral cases the volumes were also significantly less compared to the contralateral testes.

SUMMARYThe aim of this study was to evaluate testicular function in men with previous acquired undescended testis (UDT) in whom orchiopexy was performed at diagnosis compared with a similar group of men in whom spontaneous descent was awaited until puberty. Secondly, we examined the influence of age at orchiopexy on fertility parameters in adult life. A total of 169 men of the 'orchiopexy at diagnosis' group and 207 men of the 'wait and see' protocol group were invited for participation. All participants underwent an andrological evaluation, including medical history, physical examination, scrotal ultrasound, determination of reproductive hormones, and semen analysis. Results were compared for men in whom orchiopexy was performed at diagnoses with men in whom spontaneous descent was awaited until puberty followed by orchiopexy in case of non-descent. In the 'orchiopexy at diagnosis' group, 63 men of whom 14 with bilateral UDT, and in the 'wait and see' protocol group, 65 men of whom 15 with bilateral UDT were included. For unilateral UDT Inhibin B was found to be significantly lower and median progressive motility was higher in men with orchiopexy at diagnosis. For bilateral UDT, semen concentration and progressive motility showed a trend toward a favorable outcome for orchiopexy at diagnosis. Age at orchiopexy being under or above 10 years of age had no significant influence on the fertility potential. The outcome of physical examination, scrotal ultrasound, endocrine function, and semen analysis indicates a compromised fertility potential in men with previous acquired UDT. None of the protocols proved to be superior. For bilateral UDT, a trend toward favorable outcome of orchiopexy at diagnosis was seen. Furthermore, age at orchiopexy did not have an influence on fertility parameters. Therefore, in our opinion a 'conservative policy' is warranted for unilateral UDT, especially because over 50% of acquired UDT descend spontaneously.

Knowledge of the physiological testicular accumulation of 18F-fluoro-2-deoxyglucose (FDG) is essential in order to discriminate between normal and pathological findings. In this study, the 18F-FDG-uptake in healthy testes of young men was assessed using positron emission tomography/computed tomography (PET/CT)-scans. A total of 40 testes of 20 men with a mean age of 26.5 ± 3.9 years were evaluated. 18F-FDG-uptake was expressed as the standardized uptake value (SUV). Testicular volume was measured on CT and PET. All scans were assessed by three researchers, one of whom assessed every scan twice. Laterality indices and inter- and intra-observer variation were evaluated. Correlation between the SUVmax and SUVpeak, between SUVmean and SUVpeak and between age and SUVpeak were assessed. Testes showed an average SUVmax of 3.42 ± 0.61, SUVpeak of 3.06 ± 0.54 and SUVmean of 2.44 ± 0.44. The average testicular volume on CT was 23.0 ± 6.4 ml, whereas on PET it was 18.0 ± 5.1 ml. Laterality indices were calculated of 0.077 ± 0.065 (SUVmax), 0.074 ± 0.066 (SUVpeak), 0.072 ± 0.063 (SUVmean), 0.245 ± 0.259 (CT), and 0.200 ± 0.188 (PET), respectively. Inter- and intra-observer reliability were found to be perfect for the SUVs (intraclass correlation coefficient [ICC] 0.992-1.0), but poor for testicular volumes (ICC 0.854-0.902). Testicular 18F-FDG uptake in young men can be measured accurately on PET/CT and shows high symmetry. Consequently, 18F-FDG PET/CT has the potential to become a useful instrument in the evaluation of the functioning of the individual testis.

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Fertility after orchidopexied undescended testes (UDT) is impaired. Although fertility parameters are known to be more favorable in unilateral cases than in bilateral cases, the exact contribution of the unilateral orchidopexied UDT to fertility is unknown. We used testicular 18F-fluoro-2-deoxyglucose (18F-FDG)-uptake assessed by positron emission tomography/computed tomography (PET/CT) to investigate the function of the orchidopexied unilateral congenital UDT, compared to its normally descended counterpart. We hypothesize that the contribution of the orchidopexied unilateral congenital UDT to fertility in adulthood is low. Eleven men who underwent orchidopexy for congenital UDT at the age of 1.9 ± 1.4 (range, 4.5 months -4.0) years were seen in follow-up at the age of 24.1 ± 2.3 (20.6-28.0) years. All underwent physical examination, testicular ultrasonography and PET/CT. Testicular 18F-FDG-uptake was expressed as the peak Standardized Uptake Value (SUVpeak). The mean SUVpeak of the orchidopexied UDT was 2.74 ± 0.48 (2.13-3.47), which was significantly lower than its counterpart (P = 0.021). Besides, there was no correlation between the testicular volume and the SUVpeak. The orchidopexied congenital UDT has been shown to be less metabolically active than its contralateral counterpart. Nevertheless, we suggest that the operated testes function to some degree.

In the Netherlands, between 1985 and 2007 secular changes in the health care of patients with sickle cell disease (SCD) have taken place, such as penicillin prophylaxis, vaccination programs and stroke prevention. We investigated the number and causes of death in a cohort of 298 SCD patients, established in 2007, before introduction of neonatal screening, to determine preventable deaths. All patients were diagnosed with SCD before the age of 18 (median age at diagnosis 5.1 y). Their vital status was determined up to January 2017. After a total follow-up period of 4565 patient years and a median time of follow-up of 15 years for all patients, 230 patients (77%) were still alive, 45 patients (15%) were lost to follow-up and a total of 23 patients (8%) had died. Estimated survival to 18 years was 92% with a global mortality rate of 0.48 deaths/100 patient years. Leading causes of death were infection (35%) followed by neurologic complications (22%) and death in the course of a painful episode (13%). Nine of the 20 known causes of death were preventable. These results strongly suggest the benefit of comprehensive care measures for patients with SCD in the Netherlands to further prevent morbidity and mortality.