Background: Gaucher disease (GD) is an autosomal recessive disorder characterized by excessive accumulation of glucosylceramide in multiple organs. This study was performed to determine the detection rate of GD in a selected patient population with unexplained splenomegaly in Korea. Methods: This was a multicenter, observational study conducted at 18 sites in Korea between December 2016 and February 2020. Adult patients with unexplained splenomegaly were enrolled and tested for β-glucosidase enzyme activity on dried blood spots (DBS) and in peripheral blood leukocytes. Mutation analysis was performed if the test was positive or indeterminate for the enzyme assay. The primary endpoint was the percentage of patients with GD in patients with unexplained splenomegaly. Results: A total of 352 patients were enrolled in this study (male patients, 199; mean age, 48.42 yr). Amongst them, 14.77% of patients had concomitant hepatomegaly. The most common sign related to GD was splenomegaly (100%), followed by thrombocytopenia (44.32%) and, anemia (40.91%). The β-glucosidase activity assay on DBS and peripheral leukocytes showed abnormal results in sixteen and six patients, respectively. Eight patients were tested for the mutation, seven of whom were negative and one patient showed a positive mutation analysis result. One female patient who presented with splenomegaly and thrombocytopenia was diagnosed with type 1 GD. The detection rate of GD was 0.2841% (exact 95% CI, 0.0072‒). Conclusion The detection rate of GD in probable high-risk patients in Korea was lower than expected.However, the role of hemato-oncologists is still important in the diagnosis of GD.

Purpose: This study is qualitative study using phenomenology approach of Colazzi, to identify meaning and essential structure of the hospitalization with Complex Regional Pain Syndrome (CRPS). Methods: Experiential data were collected through in-depth interviews with 10 patients who had been hospitalized in hospital rehabilitation medical wards. The main question was ‘Could you describe your hospitalization experience with CRPS? Results: Five categories obtained were ‘Experience despair in the swamp of long suffering’, ‘A hospital system that only adherence to treatment procedures’, ‘There are aggravating factors all over the hospital environment’, ‘Finding support to cover the disease’, and ‘Cross the tunnel of pain with the medical staff’. Conclusion Patients with complex pain syndrome experienced extreme pain and sudden pain even after hospitalization, and were more aggravated due to treatment procedures and regulation systems. It is thought that multidisciplinary team approach nursing intervention is necessary to improve this situation.

The lower 2(nd) molar eruption is beginning to mesiolingually, then rotate to distobuccally so it has a tendency to be tilted and impacted mesially. Signs and symptoms of impacted 2(nd) molar are similar to impacted 3(rd) molar's. However, treatment plan for impacted 2nd molar is different from that of impacted 3(rd)'s. The former is the preservation and uprighting of 2(nd) molar so that it could act to recovery of mastication, symmetrical facial growth, maintaining the symmetry of dental arch, stable occlusion, while the latter is the extraction of tooth. If the uprighting treatment is planned, most proper protocol of treatment and the additional treatment opition should be applied with consideration for it's crown exposure, present of 3(rd) molar which interrupt the uprighting process, extrusion of opposite tooth. Although it could not improve the esthetic result, it could prevent many dental problems. Therefore, uprighting for impacted lower 2(nd) molar is meaningful treatment.
Crowns ; Dental Arch ; Mastication ; Molar* ; Tooth ; Tooth Movement

Chronic atherosclerotic occlusion of the abdominal aorta is a rare disease in clinical practice. In this disease, juxtarenal aortic occlusion is defined as the condition extended, adjacent to the renal arteries. The treatment of juxtarenal aortic occlusion is more difficult than a 'simple' abdominal aortic occlusion. Vascular surgery of a juxtarenal aortic occlusion-specifically aortic endarterectomy and bypass grafting-is a challenging procedure that almost invariably requires aortic cross-clamping above the level of the renal arteries, and may be associated with significant morbidity and mortality. With the advent of endovascular treatment, percutaneous transluminal angioplasty and stent implantation have been used increasingly as an alternative to conventional surgery in the management of patients with aortoiliac disease. However, endovascular treatment for juxtarenal aortic occlusion is not common and, also, special attention is needed with regard to possible renal complications. Here, we report the successful revascularization of a case of chronic juxtarenal aortic occlusion with endovascular treatment and adjunctive anticoagulation.
Angioplasty ; Aorta, Abdominal ; Endarterectomy ; Endovascular Procedures ; Humans ; Leriche Syndrome ; Rare Diseases ; Renal Artery ; Stents

Primary mediastinal leiomyosarcoma is an extremely rare neoplasm that arises from smooth muscle, usually in the esophagus or main vessels, but rarely from small vessels in the soft tissues of the mediastinum. The clinical findings depend mostly on its size and the subsequent compression of neighboring structures. The treatment of choice is surgical removal. We report the case of a 78-year-old female who presented with esophageal compression symptoms due to a 9-cm inhomogeneous mass in the posterior mediastinum. A computed tomography (CT)-guided gun biopsy and immunohistochemistry established the pathological diagnosis of leiomyosarcoma with a high grade of malignancy. Surgery was performed because of local invasion involving the aorta and diaphragm. The literature is reviewed and the pathology discussed
Aged ; Aorta ; Biopsy ; Diaphragm ; Esophagus ; Female ; Humans ; Immunohistochemistry ; Leiomyosarcoma ; Mediastinum ; Muscle, Smooth

Peripheral nerve injuries are a commonly encountered clinical problem and often result in a chronic pain and severe functional deficits. c-Fos expression is sometimes used as a marker of increased neuronal activity. We have developed herbal bath "HAC" for pain control using the following herbs: Harpagophytum procumbens, Atractylodes japonica, and Corydalis tuber. In the present study, we investigated the effects of herbal bath "HAC" on the recovery rate of the locomotor function and the expression of c-Fos in the ventrolateral periaqueductal gray (vlPAG) region of brain following sciatic crushed nerve injury in rats. Walking track analysis for the evaluation of functional recovery and immunohistochemistry for the c-Fos expression were used for this study. In the present results, characteristic gait change with dropping of the sciatic function index (SFI) was observed and c-Fos expression in the vlPAG was suppressed following sciatic crushed nerve injury in rats. Immersion into herbal bath "HAC" enhanced SFI value and restored c-Fos expression in the vlPAG to the control value. These results suggest that herbal bath "HAC" might activate neurons in the vlPAG, and it facilitates functional recovery from peripheral nerve injury. Here we showed that herbal bath "HAC" could be used as a new therapeutic intervention for pain control and functional recovery from peripheral nerve injury.
Animals ; Atractylodes ; Baths ; Brain ; Chronic Pain ; Corydalis ; Gait ; Harpagophytum ; Immersion ; Immunohistochemistry ; Neurons ; Periaqueductal Gray ; Peripheral Nerve Injuries ; Rats ; Track and Field ; Walking

Purpose: Stress hyperglycemia is common in critically ill adult patients. It is known as a predictor of increased mortality, and intensive insulin therapy has been shown to improve the prognosis in such patients. We have investigated the relationship between early stress hyperglycemia and clinical outcomes in preterm infants. Methods: In this study, 141 preterm infants with a gestational age of less than 30 weeks were enrolled. The hyperglycemic group was defined as that having maximum glucose of more than 150 mg/dL (n=61) during the first 48 h of life, and the non-hyperglycemic group was defined as that having maximum glucose of less than 150 mg/dL (n=80). Perinatal history, severity of illness using the Clinical Risk Index for Babies (CRIB) score, clinical outcomes, and mortality of the two groups were compared. Results: There was no significant difference in the gestational age between the two groups, but the birth weight (P<0.001) was significantly lower, and the CRIB score (P<0.001) was significantly higher in the hyperglycemic group. Disseminated intravascular coagulation (P<0.001) and clinically suspected sepsis (P=0.046) were more common in the hyperglycemic group. Mortality was markedly higher in the hyperglycemic group (11.3% vs. 41.0%, P<0.001). On performing a stepwise multiple logistic regression analysis, hyperglycemia (OR 3.787; 95% CI 1.324 to 10.829), the CRIB score (OR 1.252; 95% CI 1.047 to 1.496) and birth weight (OR 0.997; 95% CI 0.994 to 1.000) was independently associated with higher mortality. Conclusion: Stress hyperglycemia within the first 48 h of life is independently related to increased morbidity and mortality in preterm infants.
Adult ; Birth Weight ; Critical Illness ; Disseminated Intravascular Coagulation ; Gestational Age ; Glucose ; Humans ; Hyperglycemia ; Infant Equipment ; Infant, Newborn ; Infant, Premature ; Insulin ; Logistic Models ; Prognosis ; Sepsis

Amygdalin is known as vitamain B17, and it was called laetrile. Amygdalin is composed of two molecules of glucose, one molecule of benzaldehyde which induces an analgesic action, and one molecule of hydrocyanic acid which is an anti-neoplastic compound. Amygdalin had been used to treat cancers and relieve pain. In order to evaluate whether the analgesic action of amygdalin is related with descending pain control system, we performed patch clamp study. In the present study, the modulatory effects of amygdalin on glycine- and glutamate-induced ion currents in periaqueductal gray (PAG) neurons were investigated using the nystatin-perforated patch clamp method. Continuous application of lipopolysaccharides (LPS) on PAG neurons resulted in increased glycine-induced ion current, and in decreased glutamate-induced ion current. In contrast, continuous application of amygdalin with LPS resulted in decreased glycine-induced ion current increased by LPS, and increased glutamate- induced ion current decreased by LPS in concentration- and time-dependent fashion. These results demonstrate that amygdalin modulates neuronal activity of PAG by modulation of glycine and glutamate. Based on the present results, it can be suggested that amygdalin participates in the regulation of the descending pain control system in the level of PAG neurons. The present study demonstrated that activation of the descending pain control system is one of the possible analgesic mechanisms of amygdalin.
Amygdalin ; Animals ; Benzaldehydes ; Cyclooxygenase 2 ; Glucose ; Glutamic Acid ; Glycine ; Hydrogen Cyanide ; Lipopolysaccharides ; Neurons ; Periaqueductal Gray ; Rats

Shenqi-wan, Oriental herbal medicine formulation, has been traditionally used for delayed mental and physical development in children, complications of diabetes, and glomerulonephritis. In the present study, the protective effect of the aqueous extract of Shenqi-wan against traumatic brain injury (TBI) in the rat hippocampal dentate gyrus was investigated. For this study, step-down avoidance task, terminal deoxynuclotidyl transferase (TdT)-mediated dUTP nick end labeling (TUNEL) assay, Bax immunohistochemistry, and 5-bromo-2'-deoxyuridine (BrdU) immunohistochemistry were conducted. In the present results, it was shown that apoptotic cell death and cell proliferation in the dentate gyrus were significantly increased following TBI in rats and that the aqueous extract of Shenqi-wan suppressed the TBI-induced increase in apoptosis and cell proliferation in the dentate gyrus. Based on the present results, it is possible that the aqueous extract of Shenqi-wan has a neuroprotective effect on TBI-induced neuronal cell death.
Animals ; Apoptosis ; Brain ; Brain Injuries ; Bromodeoxyuridine ; Cell Death ; Cell Proliferation ; Child ; Dentate Gyrus ; Glomerulonephritis ; Herbal Medicine ; Humans ; Immunohistochemistry ; Neurons ; Neuroprotective Agents ; Rats ; Transferases

Gynaecotyla squatarolae (Digenea: Microphallidae) adult flukes were recovered from experimental chicks at day 4-6 post-infection and their tegumental ultrastructure was observed with a scanning electron microscopy. They were pyriform in shape, and their anterior halves were concaved ventrally. The whole body surface was covered with tegumental spines, which were wide and 16-17 digitated between oral and ventral suckers. The density of spines and number of digits decreased posteriorly. The oral sucker was subterminal and the excretory pore was at the posterior end of the worm. wo ventral suckers were similar in appearance and protruded near midline of the worm. The genital atrium was dextral to the small ventral sucker. The dorsal surface was covered with tegumental spines, but the spines were sparser than on the ventral surface. On the middle portion of the dorsal surface, a small opening presumed to be the Laurer's canal was een. From these findings, it has been confirmed that the adult G. squatarolae has unique characteristics in the surface ltrastructure.
Animals ; Brachyura/*parasitology ; Chickens ; Microscopy, Electron, Scanning ; Trematoda/anatomy & histology/classification/*isolation & purification/*ultrastructure ; Trematode Infections/*parasitology