Tolvaptan reduces height-adjusted total kidney volume (htTKV) and renal function decline in autosomal dominant polycystic kidney disease (ADPKD). This study was aimed at investigating the efficacy and safety of tolvaptan in Korean patients with ADPKD during the titration period. Methods: This study is a multicenter, single-arm, open-label phase 4 study. We enrolled 108 patients with ADPKD (age, 19–50 years) with an estimated glomerular filtration rate (eGFR) of >30 mL/min/1.73 m2 and factors defined as indicative of rapid disease progression. After tolvaptan titration, we evaluated efficacy and side effects and assessed factors associated with the effects. Results: After titration for 4 weeks, eGFR and htTKV decreased by 6.4 ± 7.9 mL/min/1.73 m2 and 16 ± 45 mL/m, respectively. No serious adverse drug reactions were observed during the titration period. The greatest eGFR decline was observed in the first week, with a starting tolvaptan dose of 45 mg. Multivariate linear regression for htTKV decline showed that the greater the change in urine osmolality (Uosm), the greater the decrease in htTKV (β, 0.436; p = 0.009) in the 1D group stratified by the Mayo Clinic image classification. Higher baseline eGFR was related to a higher htTKV reduction rate in the 1E group (β, –0.642; p = 0.009). Conclusion: We observed short-term effects and safety during the tolvaptan titration period. The decline of htTKV can be predicted as a short-term effect of tolvaptan by observing Uosm changes from baseline to end of titration in 1D and baseline eGFR in 1E groups.

A 63-year-old male presented with a painful skin lesion on the left side of the neck and upper chest approximately two months prior to presentation. Diffuse erythematous to purplish-colored sclerotic patches were observed. He had been treated with intravenous antibiotics for two weeks for cellulitis, but the lesion did not improve. Punch biopsy, and neck computed tomography (CT) with contrast enhancement were performed to differentiate between cellulitis and scleroderma. Histopathological examination revealed infiltration of pleomorphic and poorly differentiated tumor cells extending into thickened collagen bundles, and mitotic activity. Based on histologic and radiologic findings, the patient was suspected to have poorly differentiated carcinoma, and further evaluation of the origin of the carcinoma was performed. A subareolar mass on the left breast was observed on chest CT, and a needle biopsy was performed; results were consistent with findings from the skin biopsy. Finally, the patient was diagnosed with carcinoma en cuirasse, a subtype of cutaneous metastasis of breast cancer, was transferred to oncology, and underwent palliative chemotherapy.

Background: Folliculitis decalvans and dissecting cellulitis are types of primary neutrophilic cicatricial alopecia characterized by permanent hair loss. Clinicopathological differentiation is poorly described in literature. Objective: This study aimed to determine the clinicopathological distinction between folliculitis decalvans and dissecting cellulitis. Methods: A retrospective review was conducted in 45 patients diagnosed with dissecting cellulitis and folliculitis decalvans between 2011 and 2021. We reviewed the clinical features using electronic medical records, clinical photographs, and histopathologic features. Results: Clinically, middle-aged men with folliculitis decalvans showed polytrichia (80%) and papulopustules (55%), while young men with dissecting cellulitis had deeply seated nodules (84%). Histopathologically, follicular plugging was more frequently observed in dissecting cellulitis (80%) than in folliculitis decalvans (50%). There was a difference in the depth of inflammation between the two types. Conclusion A difference in clinical manifestations was observed according to the depth of inflammation. These findings may contribute to the differential diagnosis of primary neutrophilic cicatricial alopecia.

Hepatic duct diverticulum is a rare form of choledochal cyst that does not fit into the most widely used Todani classification system. Because of its rarity, it may be difficult for clinicians to diagnose and treat it. Here, we present a case of left hepatic diverticulum in a 57-year-old woman with epigastric pain. At presentation, there were mild elevations in the liver function tests. Computed tomography and magnetic resonance cholangiopancreatography showed diverticulum-like cystic lesion with sludge ball near the confluence portion of both intrahepatic bile duct, but the origin of the lesion could not be identified. The clinical impression was type II choledochal cyst. Surgical excision was planned due to recurrent abdominal pain. The operative findings revealed diverticulum arising from left hepatic duct. Histopathology confirmed the lesion to be diverticulum lined by biliary epithelium. The patient had no postoperative complication and no further symptoms since the operation.
Abdominal Pain ; Bile Ducts ; Bile Ducts, Intrahepatic ; Cholangiopancreatography, Magnetic Resonance ; Choledochal Cyst ; Classification ; Diverticulum ; Epithelium ; Female ; Hepatic Duct, Common ; Humans ; Liver Function Tests ; Middle Aged ; Postoperative Complications ; Sewage

Stress can induce a serious epileptic encephalopathy that occurs during early infancy. Recent studies have revealed that prenatal stress exposure is a risk factor for the development of infantile spasms. Our previous work demonstrates that prenatal stress with betamethasone-induced alterations to the expression of the K⁺/Cl⁻ co-transporter (KCC2) in gamma-aminobutyric acid (GABA) interneurons lowers the seizure threshold in exposed animals. Here, we further investigated the mechanisms involved in this KCC2 dysfunction and explored possible treatment options. We stressed Sprague-Dawley rats prenatally and further treated dams with betamethasone on gestational day 15, which increases seizure susceptibility and NMDA (N-Methyl-D-aspartate)-triggered spasms on postnatal day 15. In this animal model, first, we evaluated baseline calpain activity. Second, we examined the cleavage and dephosphorylation of KCC2. Finally, we checked the effect of a calpain inhibitor on seizure occurrence. The phosphorylated-N-methyl-D-aspartate Receptor 2B (NR2B):non-phosphorylated NR2B ratio was found to be higher in the cortex of the prenatally stressed beta-methasone model. We further found that the betamethasone model exhibited increased phosphorylation of calpain-2 and decreased phosphorylation of KCC2 and Glutamic acid decarboxylase 67 (GAD67). After using a calpain inhibitor in prenatal-stress rats, the seizure frequency decreased, while latency increased. GABAergic depolarization was further normalized in prenatal-stress rats treated with the calpain inhibitor. Our study suggests that calpain-dependent cleavage and dephosphorylation of KCC2 decreased the seizure threshold of rats under prenatal stress. Calpain-2 functions might, thus, be targeted in the future for the development of treatments for epileptic spasms.
Animals ; Betamethasone ; Brain Diseases ; Calpain ; Epilepsy ; gamma-Aminobutyric Acid ; Glutamate Decarboxylase ; Humans ; Infant ; Infant, Newborn ; Interneurons ; Models, Animal ; N-Methylaspartate ; Phosphorylation ; Rats ; Rats, Sprague-Dawley ; Risk Factors ; Seizures ; Spasm ; Spasms, Infantile

PURPOSE: To investigate mid-term clinical outcomes of various methods of scapholunate reconstruction for chronic scapholunate instability. METHODS: We retrospectively reviewed 11 patients diagnosed as chronic scapholunate instability, from 2005 to 2013 and followed up for more than one year after surgery. 7 patients were treated with Garcia technique of triple ligament reconstruction, 2, bone-ligamemt-bone repair, and 2, dorsal capsulodesis. Mean age was 42 years (range, 23-61 years). The right side was involved in 6 patients, and the left in 5. The follow-up period averaged 42 months (range, 13-125 months). The postoperative functional outcomes were assessed with Modified Mayo wrist score (MMWS), disability of the arm, shoulder and hand (DASH) score and range of motion. The postoperative radiologic results were analyzed with the change of scapholunate angle and distance. RESULTS: The mean MMWS score improved from 65 preoperatively to 77 postoperatively (p=0.072). The mean DASH score improved from 29.4 (range, 18.3-43.3) preoperatively to 12.7 (range, 0-38.3) postoperatively (p=0.003). The dorsiflexion increased from 67° to 78°, the volar flexion decreased from 51° to 45°, the supination increased from 69° to 88°, and the pronation increased from 62° to 66°. The SL angle changed from 55° preoperatively to 51° postoperatively, the mean SL distance changed from 3.4 mm preoperatively to 1.7 mm postoperatively. CONCLUSION: The chronic scapholunate instability is amenable to various techniques and postoperative functional outcomes improved satisfactory.
Arm ; Follow-Up Studies ; Hand ; Humans ; Ligaments* ; Pronation ; Range of Motion, Articular ; Retrospective Studies ; Shoulder ; Supination ; Wrist

BACKGROUND/AIMS: While gastric variceal bleeding (GVB) is not as prevalent as esophageal variceal bleeding, it is reportedly more serious, with high failure rates of the initial hemostasis (>30%), and has a worse prognosis than esophageal variceal bleeding. However, there is limited information regarding hemostasis and the prognosis for GVB. The aim of this study was to determine retrospectively the clinical outcomes of GVB in a multicenter study in Korea. METHODS: The data of 1,308 episodes of GVB (males:females=1062:246, age=55.0+/-11.0 years, mean+/-SD) were collected from 24 referral hospital centers in South Korea between March 2003 and December 2008. The rates of initial hemostasis failure, rebleeding, and mortality within 5 days and 6 weeks of the index bleed were evaluated. RESULTS: The initial hemostasis failed in 6.1% of the patients, and this was associated with the Child-Pugh score [odds ratio (OR)=1.619; P<0.001] and the treatment modality: endoscopic variceal ligation, endoscopic variceal obturation, and balloon-occluded retrograde transvenous obliteration vs. endoscopic sclerotherapy, transjugular intrahepatic portosystemic shunt, and balloon tamponade (OR=0.221, P<0.001). Rebleeding developed in 11.5% of the patients, and was significantly associated with Child-Pugh score (OR=1.159, P<0.001) and treatment modality (OR=0.619, P=0.026). The GVB-associated mortality was 10.3%; mortality in these cases was associated with Child-Pugh score (OR=1.795, P<0.001) and the treatment modality for the initial hemostasis (OR=0.467, P=0.001). CONCLUSIONS: The clinical outcome for GVB was better for the present cohort than in previous reports. Initial hemostasis failure, rebleeding, and mortality due to GVB were universally associated with the severity of liver cirrhosis.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Asian Continental Ancestry Group ; Endoscopy ; Esophageal and Gastric Varices/*diagnosis/mortality/therapy ; Female ; *Gastrointestinal Hemorrhage ; Humans ; Male ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Prognosis ; Republic of Korea ; Retrospective Studies ; Sclerotherapy ; Severity of Illness Index ; Treatment Outcome ; Young Adult

BACKGROUND/AIMS: Colonoscopic polypectomy should be performed on the five to seven hour of clock (standard position). However, outcomes of polypectomy at non-standard positions have not yet been investigated. This study was to compare the clinical outcomes of colonoscopic polypectomy including procedure time, status of resection margin and complications between standard and non-standard position. METHODS: Patients who underwent screening colonoscopy were prospectively recruited from Oct 2011 to Feb 2012 at Dongguk University Ilsan Hosptial, Goyang, Korea. Standard position was defined as polyps which were located from 5 to 7 hour of clock on colonoscopic view. RESULTS: A total of 168 adenomatous polyps of 114 patients were investigated. Mean size of polyp was 7.1+/-3.2 mm. The most common shape of polyps was sessile in 77 cases (45.8%). Mean overall procedure time per patient was 25.5+/-12.3 min. 130 adenomatous polyps were resected in standard position (77.4%) and 38 polyps were in non-standard position (22.6%). Overall complete resection rate was 63.7% and immediate bleeding rate was 9.8%. There was no significant difference in overall polypectomy time per polyp (2.9+/-1.3 min vs. 3.0+/-1.8 min, P=0.32). Complete resection rates and complication were not statistically different. CONCLUSIONS: There was no difference according to procedure time, status of resection margin and complications between standard position and non-standard position. Colonoscopic polypectomy is thus safe and feasible on any position.
Adenomatous Polyps ; Colonic Polyps ; Colonoscopy ; Hemorrhage ; Humans ; Korea ; Mass Screening ; Polyps ; Prospective Studies

BACKGROUND/AIMS: Ulcerative colitis (UC) is a chronic disease that characteristically has a relapsing and remitting course. Probiotics might possibly induce remission in the treatment of active UC. Aims of our study were to assess the efficacy of VSL#3 on clinical response and colonic tissue cytokine concentration changes in patients with active UC. METHODS: Twenty-four eligible patients with mild to moderate UC received open-label VSL#3 4 sachets daily in 2 divided doses for 8 weeks. The disease activity pre- and post-VSL#3 therapy was assessed by ulcerative colitis disease activity score and colonic tissue cytokine profiling done at baseline and at week 8. RESULTS: Twenty-four patients (mean age, 43.7 years; range, 20-70 years; male/female, 15/9) were enrolled and 2 patients did not have the final endoscopic assessment. A total of 22 patients were analyzed. Intent to treat analysis demonstrated remission in 45.8% of subjects (n=11); partial response in 20.8% (n=5); no change or worse in 25.0% (n=6) of subjects. The mean ulcerative colitis disease activity index (UCDAI) scores decreased from 7.09+/-1.81 to 1.45+/-1.29 in patients with a remission (p<0.001). The mean endoscopic scores had also significantly decreased from 1.91+/-0.54 to 0.63+/-0.50 in patients with a remission (p<0.001). The concentrations of colonic cytokines did not change significantly during treatment in patients with a remission. CONCLUSIONS: Our study demonstrated that VSL#3 is effective in achieving clinical responses and remissions in patients with mild-to moderately active UC, further supporting the potential role in UC therapy.
Adult ; Aged ; Colitis, Ulcerative/*therapy ; Cytokines/metabolism ; Drug Administration Schedule ; Humans ; Male ; Middle Aged ; Probiotics/*therapeutic use ; Severity of Illness Index

No abstract available.
Antiviral Agents/therapeutic use ; Ascites/diagnosis/prevention & control/therapy ; Cholagogues and Choleretics/therapeutic use ; Fatty Liver/diagnosis/diet therapy ; Fatty Liver, Alcoholic/diagnosis/drug therapy ; Hemorrhage/prevention & control/therapy ; Hepatic Encephalopathy/diagnosis/prevention & control/therapy ; Hepatitis B, Chronic/diagnosis/drug therapy ; Hepatitis C, Chronic/diagnosis/drug therapy ; Humans ; Liver Cirrhosis/*diagnosis/drug therapy/pathology/*therapy ; Liver Cirrhosis, Biliary/drug therapy ; Vasodilator Agents/therapeutic use