Fetal lower urinary tract obstruction (LUTO) is a congenital condition in which the bladder fails to excrete urine through the urethra. The primary goal of prenatal treatment for LUTO is the prevention of renal impairment and pulmonary hypoplasia. Vesico-amniotic shunt (VAS) has been the fetal intervention of choice; however, VAS has some limitations, including excretion of urine through an unphysiologic bypass and the need for postnatal corrective reoperation. In this study, we present a novel fetal intervention, a “retro-cystoscopic urethral approach,” performed on a male fetus at 20 weeks gestation diagnosed with enlarged bladder and bilateral hydronephrosis. This approach aims to dilate the narrowed urethra by inserting a urinary catheter using guidewire through a fetal cysto scope. The whole procedure was monitored under real-time ultrasonographic guidance. Despite prenatal intervention, the fetus required multiple cystocenteses, and the bladder dilation persisted.Postnatally, he was diagnosed with megacystis microcolon intestinal hypoperistalsis syndrome, a non-obstructive condition which is relatively rare in male infants. Our case emphasizes the compl exity of diagnosing LUTO during the prenatal period. Further studies exploring novel prenatal interven tions should pay more attention to candidate selection. Additionally, a thorough evaluation of organ systems beyond the urinary tract is necessary.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.

Fetal lower urinary tract obstruction (LUTO) is a congenital condition in which the bladder fails to excrete urine through the urethra. The primary goal of prenatal treatment for LUTO is the prevention of renal impairment and pulmonary hypoplasia. Vesico-amniotic shunt (VAS) has been the fetal intervention of choice; however, VAS has some limitations, including excretion of urine through an unphysiologic bypass and the need for postnatal corrective reoperation. In this study, we present a novel fetal intervention, a “retro-cystoscopic urethral approach,” performed on a male fetus at 20 weeks gestation diagnosed with enlarged bladder and bilateral hydronephrosis. This approach aims to dilate the narrowed urethra by inserting a urinary catheter using guidewire through a fetal cysto scope. The whole procedure was monitored under real-time ultrasonographic guidance. Despite prenatal intervention, the fetus required multiple cystocenteses, and the bladder dilation persisted.Postnatally, he was diagnosed with megacystis microcolon intestinal hypoperistalsis syndrome, a non-obstructive condition which is relatively rare in male infants. Our case emphasizes the compl exity of diagnosing LUTO during the prenatal period. Further studies exploring novel prenatal interven tions should pay more attention to candidate selection. Additionally, a thorough evaluation of organ systems beyond the urinary tract is necessary.

Objective: To investigate the feasibility and effects of a mobile app-based home cycling exercise program compared to home cycling exercise without additional monitoring system. Compared with fitness facilities or outdoor exercise, home-based exercise programs effectively improve physical performance in an indwelling community. However, a flexible, informal environment may decrease motivation and impair adherence to physical exercise. Mobile devices for aerobic exercise and mobile applications provide real-time monitoring, immediate feedback, and encouragement to increase motivation and promote physical performance. We investigated the feasibility and effects of a mobile app-based home exercise program on body composition, muscular strength, and cardiopulmonary function. Methods: Between February and May 2023, 20 participants were randomly allocated to the intervention (mobile application with a tablet) and control groups, and they performed aerobic exercise using a stationary bicycle for ≥150 minutes per week for 6 weeks (≤30-minute exercise session, with 3-minute warm-up and 3-minute cool-down). Karvonen formula-based heartrate defined the weekly increase in exercise intensity. Outcome measures included body-composition parameters, isokinetic knee flexor and extensor strength tests, cardiopulmonary exercise test results, and rate of target heart rate (HR) achievement. Participants were assessed at baseline and after the intervention. Results: Unrelated personal events led two participants to drop out. The intervention and control groups had similar baseline characteristics. Compared with the control group, in the post-intervention isokinetic strength test, bilateral knee flexor and extensor power, and time to target HR achievement significantly increased each week in the intervention group. Conclusion Home-based exercise to achieve long-term cardiovascular fitness with portable electronic/mobile devices facilitates individualized exercise using real-time feedback to improve motivation and adherence.

Fetal lower urinary tract obstruction (LUTO) is a congenital condition in which the bladder fails to excrete urine through the urethra. The primary goal of prenatal treatment for LUTO is the prevention of renal impairment and pulmonary hypoplasia. Vesico-amniotic shunt (VAS) has been the fetal intervention of choice; however, VAS has some limitations, including excretion of urine through an unphysiologic bypass and the need for postnatal corrective reoperation. In this study, we present a novel fetal intervention, a “retro-cystoscopic urethral approach,” performed on a male fetus at 20 weeks gestation diagnosed with enlarged bladder and bilateral hydronephrosis. This approach aims to dilate the narrowed urethra by inserting a urinary catheter using guidewire through a fetal cysto scope. The whole procedure was monitored under real-time ultrasonographic guidance. Despite prenatal intervention, the fetus required multiple cystocenteses, and the bladder dilation persisted.Postnatally, he was diagnosed with megacystis microcolon intestinal hypoperistalsis syndrome, a non-obstructive condition which is relatively rare in male infants. Our case emphasizes the compl exity of diagnosing LUTO during the prenatal period. Further studies exploring novel prenatal interven tions should pay more attention to candidate selection. Additionally, a thorough evaluation of organ systems beyond the urinary tract is necessary.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.