Addison's disease is a rare disorder resulted from a chronic deficiency of the adreanl cortical hormone. The clinical manifestations are general weakness, weight loss, hyperpigmentation, hypovolemia with hyponatremia and hyperkalemia. We report a case of Addison's disease in a 60-year-old woman who has experienced slowly progressive weakness, weight loss and generalized cutaneous pigmentation, especially sun exposed area, extensor surface and nail bed for the last, 2 years. On a hormonal assay of the adrenal glands, basal plasma cortisol level was decreased and basal plasma ACTH level was markedly elevated. A chest X-ray showed streaky tuberculous infiltration in left, upper lobe field and adrenal CT scan showed calcific densities of both adrenal glands with nodular enlargement of right adrenal gland. There was a clinical improvement with steroid replacement therapy and anti-tuber- culosis chemotherapy. A nearly normal appearance was obtained after 5 months' treatment.
Addison Disease ; Adrenal Glands ; Adrenocorticotropic Hormone ; Drug Therapy ; Female ; Humans ; Hydrocortisone ; Hyperkalemia ; Hyperpigmentation ; Hyponatremia ; Hypovolemia ; Middle Aged ; Pigmentation ; Plasma ; Solar System ; Thorax ; Tomography, X-Ray Computed ; Weight Loss

OBJECTIVE: To evaluate the feasibility and effect of ultrasound-guided ethyl alcohol injection on malleolar and olecranon synovial proliferative bursitis. METHODS: Twenty-four patients received ultrasound-guided 50% diluted ethyl alcohol injection at the site of synovial proliferative bursitis after aspiration of the free fluid. RESULTS: Swelling and symptoms significantly decreased in 13 of the 24 patients without any complications. Eleven patients had partial improvement in swelling and symptoms. CONCLUSION: Ultrasound-guided alcohol injection could be an alternative therapeutic option before surgery in patients with chronic intractable malleolar and olecranon synovial proliferative bursitis.
Bursitis* ; Ethanol* ; Humans ; Olecranon Process* ; Pilot Projects* ; Prospective Studies* ; Synovial Membrane ; Ultrasonography

Alpha-fetoprotein (AFP) is a well-known tumor marker for hepatocellular carcinoma, hepatoblastoma, and yolk sac tumors. There are several studies on AFP-producing tumors that arose from the gastrointestinal tract, pancreas, lung, kidney, and urachal tract. AFP-producing carcinoma of the gallbladder is extremely rare. We report a case of AFP-producing carcinoma of the gallbladder without liver involvement in a 58-year-old man with a gallstone, on which clinical, morphologic, and immunohistochemical studies were performed.
Male ; Humans ; Carcinoma, Hepatocellular ; Tumor Markers, Biological

PURPOSE: We investigated the safety and efficacy of peripherally inserted central catheters (PICCs) in terminally ill cancer patients. METHODS: A retrospective review was conducted on patients who underwent PICC at the hospice-palliative division of KEPCO (Korea Electric Power Corporation) Medical Center between January 2013 and December 2013. All PICCs were inserted by an interventional radiologist. RESULTS: A total of 30 terminally ill cancer patients received the PICC procedure during the study period. Including one patient who had had two PICC insertions during the period, we analyzed a total of 31 episodes of catheterization and 571 PICC days. The median catheter life span was 14.0 days (range, 1~90 days). In 25 cases, catheters were maintained until the intended time (discharge, transfer, or death), while they were removed prematurely in six other cases (19%; 10.5/1000 PICC days). Thus, the catheter maintenance success rate was 81%. Of those six premature PICC removal cases, self-removal due to delirium occurred in four cases (13%; 7.0/1000 PICC days), and catheter-related blood stream infection and thrombosis were reported in one case, each (3%; 1.8/1000 PICC days). Complication cases totaled eight (26%; 14.1/1000 PICC days). The time to complication development ranged from two to 14 days and the median was seven days. There was no PICC complication-related death. CONCLUSION: Considering characteristics of terminally ill cancer patients, such as a poor general condition, vulnerability to trivial damage, and a limited period of survival, PICC could be a safe intravenous procedure.
Catheterization ; Catheterization, Central Venous ; Catheterization, Peripheral ; Catheters* ; Delirium ; Hospice Care ; Humans ; Palliative Care ; Retrospective Studies ; Rivers ; Terminal Care ; Terminally Ill* ; Thrombosis

beta-amyloid peptide (Abeta) consisting of 40 to 42 amino acid is the principle constituent of senile plaques in Alzheimer's disease. Although, the hypothesis that deposition of AP triggers a cascade of events leading to the pathology of Alzheimer's disease has been widely accepted, direct evidence for triggering accumulation of phosphorylated tau in paired helical filament is rare. In this study, we examined neurotoxicity induced by 3 kinds of beta-amyloid peptides 1 ~28, 25~,35 and 1~40 to elucidate the way of mechanism trading to neuronal cell death caused by Abeta using cultured hippocampal neurons. For this purpose, we measured lactate dehydrogenase (LDH) in the culture media after treatment with Abeta combined with anti-oxidant drug, trolox, or not. By histochemical and TUNEL method, we studied the change of immunoreaction to anti-MAP-2 (microtubule associated protein -2, the main component of neuritis) and detected apoptotic cells, respectively, in the hippocampal neurons treated with Abeta. To investigate whether tau phosphorylation involve neurotoxicity induced by Abeta, we immunostained the neurons with anti-SMI-31 to recognize phosphorylated Ser 396/404 of tau. From our data, we suggested that Abeta1-40 and Abeta25-35 induced marked neurodegenerative changes, and the mechanism responsible for cell death caused by Abeta -neurotoxicity was associated with the apoptosis. Because Abeta-neurotoxicity was not inhibited by anti-oxidant, trolox, we suggested that anti-oxidant did not protect the neuronal cells against the damage induced by Abeta in ou. expo.imental envi.onment. Finally, we suggested that AP treatment did not potentiate the immunoreactivity to anti-phosphorylated tau antibody and we speculated that Abeta-neurotoxicity led hippocampal cells to apoptosis without tau phosphorylation.
Alzheimer Disease ; Apoptosis* ; Cell Death ; Culture Media ; In Situ Nick-End Labeling ; L-Lactate Dehydrogenase ; Neurons* ; Pathology ; Peptides* ; Phosphorylation* ; Plaque, Amyloid

PURPOSE: The purpose of this study was to explore how supervisory nurses who were transferred to a long-term care hospital from an acute hospital adapted to the new work environment. METHODS: Colaizzi's phenomenological method was applied. Participants were nine nurses working in long-term care hospitals and data were collected through in-depth individual interviews over 1 month. Approval was obtained from the Institutional Review Board (IRB). RESULTS: From this study 12 theme clusters and 5 categories were classified. The 5 categories were; ‘Unacceptable workload’, ‘Unclear job description’, ‘Unreasonable management system of the hospital’, ‘Understanding the characteristic of the long-term care hospital’ and ‘Challenge and self-development’. CONCLUSION Participants experienced conflict, identity confusion and ambivalence through the relationship with nursing assistants, long-term care workers, administrative staff and the patients’ caregivers. However, they finally understood the characteristics of the long-term care hospital and were positioned as long-term care nurses.

A 1.1 year old boy was admitted to the Seoul National University Children's Hospital because of incidental findings of hepatosplenomegaly, skin lesion and multiple intra- abdominal lymphadenopathies. Anemia and thrombocytopenia were found based on the initial complete blood count (CBC) measurements. Because of bicytopenia and hepatosplenomegaly, bone marrow examination was performed which revealed hypercellular marrow with increased monocytes and granulopoiesis. The hemoglobin F level was high for his age, and monocyte production was increased. The patient was diagnosed with juvenile myelomonocytic leukemia at the age of 1.2 years. Chemotherapy with cytarabine, etoposide, vincristine, and isotretinoin was initiated. After 6 cycles of chemotherapy, the CBC normalized. He underwent double cord blood transplantation (dCBT), but chimerism studies showed autologous recovery. However, he did not show relapse during the 5 years post-transplant during which he received isotretinoin. He is surviving disease-free 9 years after dCBT.
Anemia ; Blood Cell Count ; Bone Marrow ; Bone Marrow Examination ; Chimerism ; Cytarabine ; Drug Therapy ; Etoposide ; Fetal Blood ; Fetal Hemoglobin ; Humans ; Incidental Findings ; Isotretinoin ; Leukemia, Myelomonocytic, Juvenile ; Male ; Monocytes ; Recurrence ; Seoul ; Skin ; Thrombocytopenia ; Vincristine

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is an aggressive malignancy with a poor prognosis. DSRCT is a rare disease, and therefore a standard treatment regimen has not been established. In this study, we reviewed the clinical characteristics and treatment outcomes of pediatric DSRCT patients.METHODS: We retrospectively reviewed the medical records of 5 DSRCT patients (2 boys, 3 girls) that were diagnosed and treated with DSRCT at Seoul National University Children's Hospital from January 1999 to January 2015.RESULTS: The median age at diagnosis was 11 years 5months (range 4 years 10 months-17 years 2 months). The most frequent symptoms were abdominal pain (60%). The primary sites were gastrointestinal tract, bladder, and omentum, and the involved sites were the liver, gastrointestinal tract, bladder and bone. Three patients had multiple metastases at diagnosis. Two patients underwent upfront surgical excision of primary tumor, and the remaining 3 patients received neo-adjuvant chemotherapy after the diagnosis was confirmed by using needle biopsy. Combination chemotherapy was administered to all patients in addition to radiotherapy (median dose 45 Gy, range 17.5-54 Gy). Four patients showed disease progression or relapse, resulting in a 20% overall survival rate. At the time of analysis, one patient is alive. She had localized disease at the time of diagnosis and were treated with upfront surgery, chemotherapy, and high-dose chemotherapy with autologous stem cell transplantation and radiotherapy.CONCLUSION: Patients with DSRCT have a poor prognosis, even after multimodal treatment. Further studies are needed to determine the prognostic factors of DSRCT.
Abdominal Pain ; Biopsy, Needle ; Combined Modality Therapy ; Desmoplastic Small Round Cell Tumor ; Diagnosis ; Disease Progression ; Drug Therapy ; Drug Therapy, Combination ; Gastrointestinal Tract ; Humans ; Korea ; Liver ; Medical Records ; Neoplasm Metastasis ; Omentum ; Pediatrics ; Prognosis ; Radiotherapy ; Rare Diseases ; Recurrence ; Retrospective Studies ; Seoul ; Stem Cell Transplantation ; Survival Rate ; Treatment Outcome ; Urinary Bladder

BACKGROUND: Biodegradable polymers have increasingly been recognized for various biological applications in recent years. Here we examined the immunostimulatory activities of the novel poly(aspartic acid) conjugated with L-lysine (PLA). METHODS: PLA was synthesized by conjugating L-lysine to aspartic acid polymer. PLA-nanoliposomes (PLA-NLs) were prepared from PLA using a microfluidizer. The immunostimulatory activities of PLA-NLs were examined in mouse bone marrow-derived dendritic cells (BM-DCs). RESULTS: PLA-NLs increased the number of BM-DCs when added to cultures of GM-CSF-induced DC generation on day 4 after the initiation of cultures. Examination of the phenotypic properties showed that BM-DCs generated in the presence of PLA-NLs are more mature in terms of the expression of MHC class II molecules and major co-stimulatory molecules than BM-DCs generated in the absence of PLA-NLs. In addition, the BM-DCs exhibited enhanced capability to produce cytokines, such as IL-6, IL-12, TNF-alpha and IL-1beta. Allogeneic mixed lymphocyte reactions also confirmed that the BMDCs were more stimulatory on allogeneic T cells. PLA- NL also induced further growth of immature BM-DCs that were harvested on day 8. CONCLUSION: These results show that PLA-NLs induce the generation and functional activities of BM-DCs, and suggest that PLA-NLs could be immunostimulating agents that target DCs.
Animals ; Aspartic Acid ; Cytokines ; Dendritic Cells ; Immunomodulation ; Interleukin-12 ; Interleukin-6 ; Lymphocyte Culture Test, Mixed ; Lysine ; Mice ; Polymers ; T-Lymphocytes ; Tumor Necrosis Factor-alpha

BACKGROUND: Biodegradable polymers have increasingly been recognized for various biological applications in recent years. Here we examined the immunostimulatory activities of the novel poly(aspartic acid) conjugated with L-lysine (PLA). METHODS: PLA was synthesized by conjugating L-lysine to aspartic acid polymer. PLA-nanoliposomes (PLA-NLs) were prepared from PLA using a microfluidizer. The immunostimulatory activities of PLA-NLs were examined in mouse bone marrow-derived dendritic cells (BM-DCs). RESULTS: PLA-NLs increased the number of BM-DCs when added to cultures of GM-CSF-induced DC generation on day 4 after the initiation of cultures. Examination of the phenotypic properties showed that BM-DCs generated in the presence of PLA-NLs are more mature in terms of the expression of MHC class II molecules and major co-stimulatory molecules than BM-DCs generated in the absence of PLA-NLs. In addition, the BM-DCs exhibited enhanced capability to produce cytokines, such as IL-6, IL-12, TNF-alpha and IL-1beta. Allogeneic mixed lymphocyte reactions also confirmed that the BMDCs were more stimulatory on allogeneic T cells. PLA- NL also induced further growth of immature BM-DCs that were harvested on day 8. CONCLUSION: These results show that PLA-NLs induce the generation and functional activities of BM-DCs, and suggest that PLA-NLs could be immunostimulating agents that target DCs.
Animals ; Aspartic Acid ; Cytokines ; Dendritic Cells ; Immunomodulation ; Interleukin-12 ; Interleukin-6 ; Lymphocyte Culture Test, Mixed ; Lysine ; Mice ; Polymers ; T-Lymphocytes ; Tumor Necrosis Factor-alpha