Adrenocortical tumors in children are rare and show various clinical symptoms. We present an 8-year-old boy with peripheral precocious puberty caused by adrenocortical tumor. He showed elevated serum DHEA-S and 17-hydroxyprogesterone, and computed tomography revealed an abdominal mass. After surgical resection, he developed central precocious puberty. We report a rare case that showed progression from peripheral precocious puberty to central precocious puberty.
17-alpha-Hydroxyprogesterone ; Adrenal Cortex Neoplasms ; Child ; Humans ; Puberty, Precocious

Ophthalmoplegia may accompany herpes zoster ophthalmicus(HZO). A 60-year-old woman with rheumatoid arthritis suffered from HZO in the right side consecutively developed a mydriasis and abduction deficit in the right eye. CSF examination revealed pleocytosis and increased protein, while magnetic resonance imaging of the brain was normal. She was treated with intravenous acyclovir for 7 days. Ophthalmoplegia was completely resolved over the following 8 weeks, but mydriasis persisted.
Acyclovir ; Arthritis, Rheumatoid ; Brain ; Female ; Herpes Zoster Ophthalmicus* ; Herpes Zoster* ; Humans ; Leukocytosis ; Magnetic Resonance Imaging ; Middle Aged ; Mydriasis ; Ophthalmoplegia*

BACKGROUND: Inflammatory factors and beta-cell dysfunction due to high-fat diets aggravate chronic diseases and their complications. However, omega-3 dietary fats have anti-inflammatory effects, and the involvement of autophagy in the etiology of diabetes has been reported. Therefore, we examined the protective effects of autophagy on diabetes using fat-1 transgenic mice with omega-3 self-synthesis capability. METHODS: Streptozotocin (STZ) administration induced beta-cell dysfunction in mice; blood glucose levels and water consumption were subsequently measured. Using hematoxylin and eosin (H&E) and Masson's trichrome staining, we quantitatively assessed STZ-induced changes in the number, mass, and fibrosis of pancreatic islets in fat-1 and control mice. We identified the microtubule-associated protein 1A/1B light chain 3-immunoreactive puncta in beta-cells and quantified p62 levels in the pancreas of fat-1 and control mice. RESULTS: STZ-induced diabetic phenotypes, including hyperglycemia and polydipsia, were attenuated in fat-1 mice. Histological determination using H&E and Masson's trichrome staining revealed the protective effects of the fat-1 expression on cell death and the scarring of pancreatic islets after STZ injection. In the beta-cells of control mice, autophagy was abruptly activated after STZ treatment. Basal autophagy levels were elevated in fat-1 mice beta-cells, and this persisted after STZ treatment. Together with autophagosome detection, these results revealed that n-3 polyunsaturated fatty acid (PUFA) enrichment might partly prevent the STZ-related pancreatic islet damage by upregulating the basal activity of autophagy and improving autophagic flux disturbance. CONCLUSION: Fat-1 transgenic mice with a n-3 PUFA self-synthesis capability exert protective effects against STZ-induced beta-cell death by activating autophagy in beta-cells.
Animals ; Autophagy* ; Blood Glucose ; Cell Death ; Chronic Disease ; Cicatrix ; Diet, High-Fat ; Dietary Fats ; Drinking ; Eosine Yellowish-(YS) ; Fatty Acids, Omega-3 ; Fatty Acids, Unsaturated* ; Fibrosis ; Hematoxylin ; Hyperglycemia ; Islets of Langerhans ; Mice ; Mice, Transgenic* ; Pancreas ; Phenotype ; Polydipsia ; Streptozocin

Pseudoaneurysm is a rare life-threatening complication of chronic pancreatitis. It can be diagnosed by various imaging modalities including computerized tomography (CT), ultrasound, and angiography. Early diagnosis and radiologic or surgical treatment can promise better outcomes. However, pseudoaneurysm is not easily diagnosed. It can be misdiagnosed as a pseudocyst with secondary infection. Rarely, the correct diagnosis is made by an inadvertent trial with percutaneous drainage. The endoscopically identified hemosuccus pancreaticus is also a rare finding. Recently, we experienced two cases of pseudoaneurysm in patients with chronic pancreatitis. They did not have any evidence of bleeding in the initial endoscopy or evidence of pseudoaneurysms in the initial ultrasound and CT scan. In one case, the pseudoaneurysm was identified during a percutaneous drainage procedure, performed to diagnose and manage a cystic lesion which appeared to be an infected cyst. In the other case, the pseudoaneurysm was suspected after the hemosuccus pancreaticus was found during endoscopy performed due to recurrent hematemesis. Both cases were successfully treated with arterial embolization of the pseudoaneurysms.
Aneurysm, False* ; Angiography ; Coinfection ; Diagnosis ; Drainage* ; Early Diagnosis ; Endoscopy ; Hematemesis ; Hemorrhage ; Humans ; Pancreatitis, Chronic* ; Tomography, X-Ray Computed ; Ultrasonography

Sodium 2-mercaptoethanesulfonate (mesna) is a protective agent that is widely used in medicine because of its antioxidant effects. Recently, reactive oxygen species (ROS) were shown to increase pigmentation. Thus, ROS scavengers and inhibitors of ROS production may suppress melanogenesis. Forkhead box-O3a (FoxO3a) is an antimelanogenic factor that mediates ROS-induced skin pigmentation. In this study, we aimed to investigate the whitening effect of mesna and the signaling mechanism mediating this effect. Human melanoma (MNT-1) cells were used in this study. mRNA and protein expression were measured by real-time quantitative PCR and Western blotting analysis to track changes in FoxO3a-related signals induced by mesna. An immunofluorescence assay was performed to determine the nuclear translocation of FoxO3a. When MNT-1 melanoma cells were treated with mesna, melanin production and secretion decreased. These effects were accompanied by increases in FoxO3a activation and nuclear translocation, resulting in downregulation of four master genes of melanogenesis: MITF, TYR, TRP1, and TRP2. We found that mesna, an antioxidant and radical scavenger, suppresses melanin production and may therefore be a useful agent for the clinical treatment of hyperpigmentation disorders.

Delphinidin is a major anthocyanidin compound found in various vegetablesand fruits. It has anti-oxidant, anti-inflammatory, and various other biologicalactivities. In this study we demonstrated the anti-cancer activity of delphinidin,which was related to autophagy, in radiation-exposed non-small cell lung cancer(NSCLC). Radiosensitising effects were assessed in vitro by treating cells with a subcytotoxicdose of delphinidin (5 M) before exposure to -ionising radiation (IR). Wefound that treatment with delphinidin or IR induced NSCLC cell death in vitro; howeverthe combination of delphinidin pre-treatment and IR was more effective thaneither agent alone, yielding a radiation enhancement ratio of 1.54 at the 50% lethaldose. Moreover, combined treatment with delphinidin and IR, enhanced apoptoticcell death, suppressed the mTOR pathway, and activated the JNK/MAPK pathway.Delphinidin inhibited the phosphorylation of PI3K, AKT, and mTOR, and increasedthe expression of autophagy-induced cell death associated-protein in radiation-exposedNSCLC cells. In addition, JNK phosphorylation was upregulated by delphinidinpre-treatment in radiation-exposed NSCLC cells. Collectively, these results show thatdelphinidin acts as a radiation-sensitizing agent through autophagy induction andJNK/MAPK pathway activation, thus enhancing apoptotic cell death in NSCLC cells.

Sodium 2-mercaptoethanesulfonate (mesna) is a protective agent that is widely used in medicine because of its antioxidant effects. Recently, reactive oxygen species (ROS) were shown to increase pigmentation. Thus, ROS scavengers and inhibitors of ROS production may suppress melanogenesis. Forkhead box-O3a (FoxO3a) is an antimelanogenic factor that mediates ROS-induced skin pigmentation. In this study, we aimed to investigate the whitening effect of mesna and the signaling mechanism mediating this effect. Human melanoma (MNT-1) cells were used in this study. mRNA and protein expression were measured by real-time quantitative PCR and Western blotting analysis to track changes in FoxO3a-related signals induced by mesna. An immunofluorescence assay was performed to determine the nuclear translocation of FoxO3a. When MNT-1 melanoma cells were treated with mesna, melanin production and secretion decreased. These effects were accompanied by increases in FoxO3a activation and nuclear translocation, resulting in downregulation of four master genes of melanogenesis: MITF, TYR, TRP1, and TRP2. We found that mesna, an antioxidant and radical scavenger, suppresses melanin production and may therefore be a useful agent for the clinical treatment of hyperpigmentation disorders.

Delphinidin is a major anthocyanidin compound found in various vegetablesand fruits. It has anti-oxidant, anti-inflammatory, and various other biologicalactivities. In this study we demonstrated the anti-cancer activity of delphinidin,which was related to autophagy, in radiation-exposed non-small cell lung cancer(NSCLC). Radiosensitising effects were assessed in vitro by treating cells with a subcytotoxicdose of delphinidin (5 M) before exposure to -ionising radiation (IR). Wefound that treatment with delphinidin or IR induced NSCLC cell death in vitro; howeverthe combination of delphinidin pre-treatment and IR was more effective thaneither agent alone, yielding a radiation enhancement ratio of 1.54 at the 50% lethaldose. Moreover, combined treatment with delphinidin and IR, enhanced apoptoticcell death, suppressed the mTOR pathway, and activated the JNK/MAPK pathway.Delphinidin inhibited the phosphorylation of PI3K, AKT, and mTOR, and increasedthe expression of autophagy-induced cell death associated-protein in radiation-exposedNSCLC cells. In addition, JNK phosphorylation was upregulated by delphinidinpre-treatment in radiation-exposed NSCLC cells. Collectively, these results show thatdelphinidin acts as a radiation-sensitizing agent through autophagy induction andJNK/MAPK pathway activation, thus enhancing apoptotic cell death in NSCLC cells.

Biliary complication occurs in 6-34% of all liver transplant patients. Although bile leaks and strictures are relatively common, other biliary complications such as T-tube leak, choledocholithiasis, and biliary cast syndrome can also be observed. The biliary cast syndrome describes the presence of casts causing obstruction with its resultant sequelae of biliary infection, hepatocyte damage secondary to bile stasis and ductal damage, all contributing to cholangiopathy. Because the exact timing of cast formation after orthotopic liver transplantation is not consistent, it is difficult to define the true incidence of biliary cast syndrome without long-term follow-up data. Proposed etiological mechanisms include acute cellular rejection, prolongation of cold ischemic time, infection, biliary drainage tubes, and biliary obstruction. The diagnosis of biliary cast syndrome is usually confirmed by endoscopic retrograde cholangiopancreatography. There have been few published articles about biliary casts in Korea. Herein, we report a case of biliary cast syndrome followed by orthotopic liver transplantation.
Adult ; Bile Duct Diseases/*complications/diagnosis/etiology ; Female ; Humans ; Jaundice, Obstructive/*etiology ; Liver Transplantation/*adverse effects ; Male ; Middle Aged ; Retrospective Studies ; Syndrome

PURPOSE: To investigate the effects of radiation dose-escalation on the treatment outcome, complications and the other prognostic variables for glioblastoma patients treated with 3D-conformal radiotherapy (3D-CRT). MATERIALS AND METHODS: Between Jan 1997 and July 2002, a total of 75 patients with histologically proven diagnosis of glioblastoma were analyzed. The patients who had a Karnofsky Performance Score (KPS) of 60 or higher, and received at least 50 Gy of radiation to the tumor bed were eligible. All the patients were divided into two arms; Arm 1, the high-dose group was enrolled prospectively, and Arm 2, the low-dose group served as a retrospective control. Arm 1 patients received 63~70 Gy (Median 66 Gy, fraction size 1.8~2 Gy) with 3D-conformal radiotherapy, and Arm 2 received 59.4 Gy or less (Median 59.4 Gy, fraction size 1.8 Gy) with 2D-conventional radiotherapy. The Gross Tumor Volume (GTV) was defined by the surgical margin and the residual gross tumor on a contrast enhanced MRI. Surrounding edema was not included in the Clinical Target Volume (CTV) in Arm 1, so as to reduce the risk of late radiation associated complications; whereas as in Arm 2 it was included. The overall survival and progression free survival times were calculated from the date of surgery using the Kaplan-Meier method. The time to progression was measured with serial neurologic examinations and MRI or CT scans after RT completion. Acute and late toxicities were evaluated using the Radiation Therapy Oncology Group neurotoxicity scores. RESULTS: During the relatively short follow up period of 14 months, the median overall survival and progression free survival times were 15+/-1.65 and 11+/-0.95 months, respectively. There was a significantly longer survival time for the Arm 1 patients compared to those in Arm 2 (p=0.028). For Arm 1 patients, the median survival and progression free survival times were 21+/-5.03 and 12+/-1.59 months, respectively, while for Arm 2 patients they were 14+/-0.94 and 10+/-1.63 months, respectively. Especially in terms of the 2-year survival rate, the high-dose group showed a much better survival time than the low-dose group; 44.7% versus 19.2%. Upon univariate analyses, age, performance status, location of tumor, extent of surgery, tumor volume and radiation dose group were significant factors for survival. Multivariate analyses confirmed that the impact of radiation dose on survival was independent of age, performance status, extent of surgery and target volume. During the follow-up period, complications related directly with radiation, such as radionecrosis, has not been identified. CONCLUSION: Using 3D-conformal radiotherapy, which is able to reduce the radiation dose to normal tissues compared to 2D-conventional treatment, up to 70 Gy of radiation could be delivered to the GTV without significant toxicity. As an approach to intensify local treatment, the radiation dose escalation through 3D-CRT can be expected to increase the overall and progression free survival times for patients with glioblastomas.
Arm ; Diagnosis ; Disease-Free Survival ; Edema ; Follow-Up Studies ; Glioblastoma* ; Humans ; Magnetic Resonance Imaging ; Multivariate Analysis ; Neurologic Examination ; Prospective Studies ; Radiotherapy* ; Retrospective Studies ; Survival Rate ; Tomography, X-Ray Computed ; Treatment Outcome ; Tumor Burden