No abstract available.
Leukemia, Plasma Cell* ; Plasma Cells* ; Plasma* ; Plasmacytoma*

OBJECTIVE: This study was designed to evaluate the cause of mortality in patients with dementia, including Alzheimer's disease and multi-infarct dementia, and those without dementia. METHOD: Hospital records of geriatric patients (> or =65) in nursing home having dementia were reviewed retrospectively for 6 years. The total number of patients was 351 (the number of demented patients was 146 and those without dementia was 68). Of the ones with dementia, 146 had dementia of Alzheimer's type, 101 patients had vascular dementia, and 36 patients had non-specific dementia. This study focused on the different complications associated with Alzheimer's disease and cerebrovascular dementia. RESULT: 1) The average age at death of demented patient is higher than the non-demented population;however, there is no difference in age between Alzheimer's disease and vascular dementia. 2) There is no difference in time spent in the nursing home among the non-demented group, Alzheimer's disease and vascular dementia group from admission to death. The life expectancy of male patients with Alzheimer's disease after admission to the nursing home is significant shorter than their female counterpart. 3) Patients with vascular dementia have a higher incidence of hypertension, atherosclerotic disease, and diabetes than the Alzheimer's patients. 4) The main overall causes of the death (in nursing home patients) are infection and sepsis. In Alzheimer's disease's, sepsis is the major cause of death, while in vascular dementia, atherosclerotic disease is the number one cause. CONCLUSION: Knowledge of the causes of the death in demented patients may help the management of the possible complications associated with the different types of dementia. However, long-term research is then needed to analyze the survival rates of demented nursing home patients.
Alzheimer Disease ; Cause of Death ; Dementia* ; Dementia, Multi-Infarct ; Dementia, Vascular ; Female ; Hospital Records ; Humans ; Hypertension ; Incidence ; Life Expectancy ; Male ; Mortality* ; Nursing Homes* ; Nursing* ; Retrospective Studies ; Sepsis ; Survival Rate

BACKGROUND: Here we investigated the clinical utilities of blast suspect, large unstained cell (LUC), delta neutrophil index ll (DN ll), and delta neutrophil index l (DN l), analyzed in peripheral blood samples with automated hematology analyzers to predict the relapse of acute leukemia. METHODS: We retrospectively reviewed the medical records of 112 patients, including 56 patients with acute leukemia relapse and 56 controls. Blast suspect, LUC, DN ll, and DN l were compared between the control and leukemia relapse groups. RESULTS: Significant differences in blast suspect (P<0.001), LUC (P<0.001), DN ll (P<0.001), and DN l (P=0.002) were observed between the leukemia relapse and control groups. The areas under the curve (AUC) value was 0.927 for blast suspect (95% confidence interval [CI]: 0.8750.978, P<0.001), 0.868 for LUC (95% CI: 0.794–0.941, P<0.001), and 0.900 for DN ll (95% CI: 0.841–0.960, P<0.001). Logistic regression analysis for the prediction of leukemia relapse revealed odds ratio values of 1.52 (95% CI: 1.26–1.96, P=0.0002) for blast suspect, 1.66 (95% CI: 1.27–2.42, P=0.0019) for LUC, 1.16 (95% CI: 1.08–1.29, P=0.0014) for DN ll, and 1.05 (95% CI: 1.01–1.13, P=0.0845) for DN l. CONCLUSIONS: Multiple parameters provided by automated blood cell analyzers may serve as powerful ancillary tools for the prediction and diagnosis of leukemia relapse.
Blood Cells ; Diagnosis ; Hematology ; Humans ; Leukemia ; Logistic Models ; Medical Records ; Neutrophils ; Odds Ratio ; Recurrence ; Retrospective Studies

Peptic ulcer disease is the most common cause of acute gastrointestinal bleeding, followed by variceal bleeding, Mallory–Weiss syndrome, and malignancy. On the contrary, acquired hemophilia A is a very rare hemorrhagic disease, which usually manifests with musculocutaneous bleeding, caused by autoantibodies against coagulation factor VIII.
A 78-year-old man presented to the Emergency Department with melena. Dieulafoy’s lesions were observed on esophagogastroduodenoscopy, and endoscopic cauterization was performed. However, the patient complained of back pain and symptoms indicative of upper gastrointestinal bleeding. Abdominopelvic computed tomography was performed, and hematoma in the psoas muscle was detected. Antibodies against coagulation factor VIII were confirmed with a blood test, and the diagnosis of acquired hemophilia A was made. Here, we report a case of acquired hemophilia A presenting with upper gastrointestinal bleeding symptoms and present a brief review of literature.

PURPOSE: To investigate the production of long pentraxin 3 (PTX3) in response to tunicamycin-induced endoplasmic reticulum (ER) stress and its role in ER stress-associated cell death, PTX3 expression was evaluated in the human retinal pigment epithelial cell line, ARPE-19. METHODS: PTX3 production in ARPE-19 cells was analyzed in the absence or presence of tunicamycin treatment by enzyme-linked immunosorbent assay. PTX3 protein and mRNA levels were estimated using western blot analysis and real-time reverse transcription-polymerase chain reaction, respectively. Protein and mRNA levels of CCAAT-enhancer-binding protein homologous protein (CHOP) and ARPE-19 cell viability were measured in the presence of tunicamycin-induced ER stress in control or PTX3 small hairpin RNA (shRNA)-transfected ARPE-19 cells. RESULTS: The protein and mRNA levels of PTX3 were found to be significantly increased by tunicamycin treatment. PTX3 production was significantly decreased in inositol-requiring enzyme 1α shRNA-transfected ARPE-19 cells compared to control shRNA-transfected cells. Furthermore, pretreatment with the NF-κB inhibitor abolished tunicamycin-induced PTX3 production. Decreased cell viability and prolonged protein and mRNA expression of CHOP were observed under tunicamycin-induced ER stress in PTX3 shRNA transfected ARPE-19 cells. CONCLUSIONS: These results suggest that PTX3 production increased in the presence of tunicamycin-induced ER stress. Therefore, PTX3 could be an important protector of ER stress-induced cell death in human retinal pigment epithelial cells. Inositol-requiring enzyme 1α and the NF-κB signaling pathway may serve as potential targets for regulation of PTX3 expression in the retina. Therefore, their role in PTX3 expression needs to be further investigated.
Anti-Bacterial Agents/pharmacology ; Apoptosis ; Blotting, Western ; C-Reactive Protein/biosynthesis/*genetics ; Cells, Cultured ; Endoplasmic Reticulum Stress/*drug effects/genetics ; Enzyme-Linked Immunosorbent Assay ; *Gene Expression Regulation ; Humans ; Polymerase Chain Reaction ; RNA, Messenger/*genetics ; Retinal Pigment Epithelium/*metabolism/pathology ; Serum Amyloid P-Component/biosynthesis/*genetics ; Tunicamycin/*pharmacology

In patients with acute myeloid leukemia (AML), pleural effusion may be attributed to various factors, including infection, hypoalbuminemia, and renal failure. However, leukemic infiltration of the pleural fluid is rarely reported and poorly understood. Extramedullary diseases have been reported with increasing frequency as the survival rates of patients with AML have increased. However, the reported prognostic effects of leukemic pleural effusion in patients with AML range from none to a worse prognosis. Here, we report a case of acute promyelocytic leukemia (APL) in a patient exhibiting leukemic pleural effusion with fluorescence in situ hybridization (FISH) results indicating the presence of the PML-RARA fusion gene. A 52-year-old man presented with pancytopenia, dyspnea, and fever. He had a medical history of hypertension, end-stage renal disease, and hepatitis B virus-related liver cirrhosis. A peripheral blood smear revealed the presence of multiple abnormally hypergranular promyelocytes. White blood cell differential counts were not performed due to severe pancytopenia. A bone marrow examination, immunophenotyping analysis, and cytogenetic and molecular studies revealed APL. The patient was treated with all-trans retinoic acid immediately after abnormal promyelocytes were observed in the peripheral blood smear, but induction chemotherapy was delayed because of his poor condition. His persistent dyspnea and abdominal discomfort led to a thoracentesis and the observation of abnormal promyelocytes that were positive for PML-RARA fusion gene by FISH. To our knowledge, this is the first report of leukemic pleural infiltration with PML-RARA fusion gene-positivity via FISH.
Bone Marrow Examination ; Cytogenetics ; Dyspnea ; Fever ; Fluorescence ; Granulocyte Precursor Cells ; Hepatitis B ; Humans ; Hypertension ; Hypoalbuminemia ; Immunophenotyping ; In Situ Hybridization ; Induction Chemotherapy ; Kidney Failure, Chronic ; Leukemia, Myeloid, Acute ; Leukemia, Promyelocytic, Acute ; Leukemic Infiltration ; Leukocytes ; Liver Cirrhosis ; Middle Aged ; Pancytopenia ; Pleural Effusion ; Prognosis ; Renal Insufficiency ; Survival Rate ; Thoracentesis ; Tretinoin

Sacrococcygeal teratoma is the most common congenital tumor in neonates, and is reported in approximately 1/35,000 to 1/40,000 live births. Oligodendroglioma is a rare central nervous system tumor that is usually found in the cerebral hemisphere of young and middle aged adults. When associated with a teratoma, it is mainly identified in ovarian teratoma in adolescents and adults. We describe a rare case of a preterm infant with oligodendroglioma in a mature sacrococcygeal teratoma. The male neonate was born at a gestational age of 30 weeks with a protruding mass in the sacrococcygeal region. Pelvic magnetic resonance imaging showed a sacrococcygeal teratoma of approximately 11 cm comprising fat components and skeletal structure, that extended from the anterior part of the sacrum to the abdominal cavity. Radical resection was performed at 36 days of age. Macroscopically, the resected intra-abdominal mass had the characteristics of a cystic lesion, and the intrapelvic mass was a predominantly solid mixed cystic-solid lesion. Histologically, this solid lesion in the intrapelvic mass was composed of mature glial tissue, which comprised as a proliferation of monotonous cells with small and round nuclei, surrounded by a perinuclear halo (“fried egg” appearance). Additionally, these cells were immunohistochemically positive for glial fibrillary acidic protein. These findings confirmed the diagnosis of oligodendroglioma in sacrococcygeal mature teratoma. After the treatment, no recurrence was observed during the follow-up period, and no additional intervention was required. However, the patient is undergoing treatment for voiding dysfunction caused by a neurogenic bladder.

Internal hernia (IH) is a rare cause of small bowel obstruction occurs when there is protrusion of an internal organ into a retroperitoneal fossa or a foramen in the abdominal cavity. IH can be presented with acute or chronic abdominal symptom and discovered by accident in operation field. However, various kinds of imaging modalities often do not provide the assistance to diagnose IH preoperatively, but computed tomography (CT) scan has a high diagnostic accuracy. We report a case of congenital IH in a 6-year-old boy who experienced life threatening shock. CT scan showed large amount of ascites, bowel wall thickening with poor or absent enhancement of the strangulated bowel segment. Surgical exploration was performed immediately and had to undergo over two meters excision of strangulated small bowel. To prevent the delay in the diagnosis of IH, we should early use of the CT scan and take urgent operation.
Abdominal Cavity ; Ascites ; Child ; Hernia ; Humans ; Intestinal Obstruction ; Shock

Non-infectious complications of peritoneal dialysis (PD) are relatively less common than infectious complications but are a potentially serious problem in patients on chronic PD. Here, we present a case of a non-infectious complication of PD in a 13-year-old boy on chronic PD who presented with symptoms such as hypertension, edema, dyspnea, and decreased ultrafiltration. Chest and abdominal radiography showed pleural effusion and migration of the PD catheter tip. Laparoscopic PD catheter reposition was performed because PD catheter malfunction was suspected. However, pleural effusion relapsed whenever the dialysate volume increased. To identify peritoneal leakage, computed tomography (CT) peritoneography was performed, and a defect of the peritoneum in the left lower abdomen with contrast leakage to the left rectus and abdominis muscles was observed. He was treated conservatively by transiently decreasing the volume of night intermittent PD and gradually increasing the volume. At the 2-year follow-up visit, the patient had not experienced similar symptoms. Patients on PD who present with refractory or recurrent pleural effusion that does not respond to therapy should be assessed for the presence of infection, catheter malfunction, and pleuroperitoneal communication. Thoracentesis and CT peritoneography are useful for evaluating pleural effusion, and timely examination is important for identifying the defect or fistula.
Abdomen ; Adolescent ; Catheters ; Dyspnea ; Edema ; Fistula ; Follow-Up Studies ; Humans ; Hypertension ; Male ; Muscles ; Peritoneal Dialysis ; Peritoneum ; Pleural Effusion ; Radiography, Abdominal ; Thoracentesis ; Thorax ; Ultrafiltration

Background: Despite the popularity of the NeoBase 2 Non-derivatized MSMS assay (PerkinElmer, Turku, Finland), there are no reports of its comprehensive evaluation, including the ability to distinguish transient tyrosinemia of the newborn (TTN) from tyrosinemia type 1 (TYR 1) using succinylacetone (SUAC). No newborn screening (NBS) cutoffs for preterm neonates in the Korean population have been suggested. We evaluated the NeoBase 2 assay and identified analytes requiring different cutoffs in preterm neonates. Methods: Residual NBS dried blood spot samples and proficiency testing (PT) materials of the Newborn Screening Quality Assurance Program and the Korean Association of External Quality Assessment Service were used. Precision, accuracy, limit of detection (LOD), lower limit of quantification (LLOQ), linearity, recovery, carryover, and performance of SUAC were evaluated. Cutoffs were determined, and analytes requiring different cutoffs in preterm neonates were investigated. Results: Mean CVs for within-run and between-day precision were within 15%. Accuracy analysis indicated high agreement with in-house derivatized assay results and results of other PT participants. All analytes demonstrated acceptable LOD, LLOQ, and linearity. Recoveries were acceptable, except for SUAC. Carryover was negligible. Cutoffs were established for all analytes; Tyr, adenosine, and C20:0-lysophosphatidylcholine required different cutoffs in preterm neonates. Differential diagnosis of TYR 1 and TTN was successful with simultaneous Tyr and SUAC measurement. Conclusions The NeoBase 2 assay demonstrated satisfactory performance. The additional analytes provide a wider diagnostic coverage, and the simultaneous measurement of Tyr and SUAC is efficient in excluding TYR 1. The new cutoffs for preterm neonates may decrease false-positive rates, without compromising diagnostic sensitivity.