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.

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.

Coronavirus disease 2019 (COVID-19) is a global pandemic that had affected more than eight million people worldwide by June 2020. Given the importance of the presence of diabetes mellitus (DM) for host immunity, we retrospectively evaluated the clinical characteristics and outcomes of moderate-to-severe COVID-19 in patients with diabetes.

BACKGROUND AND PURPOSE: To determine the correlation between the cross-sectional area (CSA) of the median nerve measured at the wrist using three-dimensional (3D) ultrasonography (US) and the electrophysiological severity of carpal tunnel syndrome (CTS). METHODS: We prospectively examined 102 wrists of 51 patients with clinical CTS, which were classified into 3 groups according to the electrodiagnostic (EDX) findings. Median nerve CSAs were measured using 3D US at the carpal tunnel inlet and at the level of maximal swelling. RESULTS: Ten wrists were negative for CTS. Of the 92 CTS-positive wrists, 23, 30, and 39 were classified as having mild, moderate, and severe CTS, respectively. The median nerve CSA differed significantly between the severe- and moderate-CTS groups (p=0.0007 at the carpal tunnel inlet and p<0.0001 at the maximal swelling site). There was a correlation between median nerve CSA and EDX parameters among those wrists with severe and mild CTS (p<0.0001 at both sites). CONCLUSIONS: The median nerve CSA as measured by 3D US could provide additional information about the severity of CTS, as indicated by the strong correlation with standard EDX findings.
Bays ; Carpal Tunnel Syndrome* ; Humans ; Median Nerve ; Prospective Studies ; Ultrasonography* ; Wrist

Transvenous left ventricular (LV) lead implantation is on the increase due to cardiac resynchronization therapy (CRT). However, there has been paucity of data on the prognosis of LV lead. Consecutive 32 patients with LV lead for CRT (n=22) or pacemaker (n=10) were subjected. Serial changes in pacing threshold and impedance along with lead-related complications were evaluated. Over 2 yr follow-up, there was no significant change in relative threshold voltage to the initial value (100%, 110%, 89.6%, and 79.6% at baseline, 1, 6, and 24 months respectively, P=0.62) as well as lead impedance (816+/-272, 650+/-178, 647+/-191, and 590+/-185 ohm at baseline, 1, 6, and 24 months respectively, P=0.80). The threshold change was not affected by lead position, lead polarity, and indication of lead implantation. The cumulative rates of lead revision were 6.3% (n=2) and 9.4% (n=3) in 6 month and 2 yr follow-up, respectively. One case of phrenic nerve capture at left lateral decubitus position was detected 1 month after the implantation. However, there were no serious complications over 2 yr period. In conclusion, transvenous LV lead implantation showed favorable long-term prognosis. Pacing parameters remained stable without significant changes over 2 yr follow-up.
Adult ; Aged ; Analysis of Variance ; Cardiac Electrophysiology ; Cardiac Resynchronization Therapy ; *Cardiac Resynchronization Therapy Devices ; Female ; Follow-Up Studies ; *Heart Ventricles ; Humans ; Male ; Middle Aged ; Multivariate Analysis ; Prognosis ; Ventricular Dysfunction, Left/therapy

Sump syndrome is a rare late complication of choledochoenteric anastomosis, and this caused by the accumulation of food debris, choledocholithiasis, bile sludge and cholesterol crystals in the distal common bile duct. This syndrome is characterized by symptoms such as abdominal pain and fever. The treatment modality for this syndrome has been surgery in the past. However, endoscopic treatment such as endoscopic sphinterotomy is currently regarded as the primary therapeutic approach for this condition. We experienced a patient with a history of choledochoduodenostomy and who developed sump syndrome as a complication of the surgery. Endoscopic sphinterotomy was performed for treatment, but this only produced the recurrence of the disease. The recurrent sump syndrome was eventually successfully controlled by performing endoscopic papillary balloon dilatation.
Abdominal Pain ; Bile ; Choledocholithiasis ; Choledochostomy ; Cholesterol ; Common Bile Duct ; Dilatation ; Fever ; Humans ; Postcholecystectomy Syndrome ; Recurrence ; Sewage

Wegener's granulomatosis (WG) is a disease characterized by a granulomatous necrotizing vasculitis of small vessels. Although any organ systems can be involved, gastrointestinal involvement in WG is notably uncommon. We present a case of 67-year-old man who was admitted with abdominal pain and diarrhea lasting for 3 weeks. Colonoscopy and abdominal CT scan revealed vasculitis and multiple mesenteric lymphadenopathy. Jejunum and mesenteric lymph nodes biopsies confirmed limited form of WG. The present case indicates that WG might involve only gastrointestinal tract and the histological confirmation is important for diagnosis.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Colonoscopy ; Diagnosis, Differential ; Gastroenteritis/complications/*diagnosis ; Humans ; Lymphatic Diseases/complications/*diagnosis/pathology ; Male ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed ; Wegener Granulomatosis/complications/*diagnosis/drug therapy

Wegener's granulomatosis (WG) is a disease characterized by a granulomatous necrotizing vasculitis of small vessels. Although any organ systems can be involved, gastrointestinal involvement in WG is notably uncommon. We present a case of 67-year-old man who was admitted with abdominal pain and diarrhea lasting for 3 weeks. Colonoscopy and abdominal CT scan revealed vasculitis and multiple mesenteric lymphadenopathy. Jejunum and mesenteric lymph nodes biopsies confirmed limited form of WG. The present case indicates that WG might involve only gastrointestinal tract and the histological confirmation is important for diagnosis.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Colonoscopy ; Diagnosis, Differential ; Gastroenteritis/complications/*diagnosis ; Humans ; Lymphatic Diseases/complications/*diagnosis/pathology ; Male ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed ; Wegener Granulomatosis/complications/*diagnosis/drug therapy

The pharmacokinetics and dosage regimen of norfloxacin-glycine acetate (NFLXGA) was investigated in pigs after a single intravenous (i.v.) or oral (p.o.) administration at a dosage of 7.2 mg/kg body weight. After both i.v. and p.o. administration, plasma drug concentrations were best fitted to an open two-compartment model with a rapid distribution phase. After i.v. administration of NFLXGA, the distribution (t1/2alpha) and elimination half-life (t1/2beta) were 0.36 +/- 0.07 h and 7.42 +/- 3.55 h, respectively. The volume of distribution of NFLXGA at steady state (Vdss) was 4.66 +/- 1.39 l/kg. After p.o. administration of NFLXGA, the maximal absorption concentration (Cmax) was 0.43 +/- 0.06 microgram/ ml at 1.36 +/- 0.39 h (Tmax). The mean absorption (t1/2ka) and elimination half-life (t1/2beta) of NFLXGA were 0.78 +/- 0.27 h and 7.13 +/- 1.41 h, respectively. The mean systemic bioavailability (F) after p.o. administration was 31.10 +/- 15.16%. We suggest that the optimal dosage calculated from the pharmacokinetic parameters is 5.01 mg/kg per day i.v. or 16.12 mg/kg per day p.o.
Administration, Oral ; Animals ; Anti-Bacterial Agents/administration & dosage/blood/*pharmacokinetics ; Biological Availability ; Cross-Over Studies ; Glycine/administration & dosage/*analogs & derivatives/blood/pharmacokinetics ; Half-Life ; Injections, Intravenous/veterinary ; Male ; Norfloxacin/administration & dosage/*analogs & derivatives/blood/pharmacokinetics ; Swine/*metabolism ; Time Factors