We experienced two cases of Gaucher's disease of adult type in brother aged four years and two years. The patients showed hepatosplenomegaly with anemia and thrombocytopenia. Typical Gaucher cells were found in bone marrow and biopsy specimens of liver and spleen through light and electron microscopic examination. Splenectomy was followed by improvement of anemia and thrombocytopenia. A brief review of literature was made.
Adult ; Anemia ; Biopsy ; Bone Marrow ; Gaucher Disease* ; Humans ; Liver ; Siblings* ; Spleen ; Splenectomy ; Thrombocytopenia

Myocardial bridging is defined as segmental engulfment of a major epicardial coronary artery by myocardial fibers, causing a systolic narrowing or milking effect of the coronary arterial segment. During systole, the intramuscular part of coronary artery is compressed by contraction of overbridging ventricular muscle, therefore blood flow distal to the lesion is impaired and angina pectoris or acute myocardial infarction may occur. We experienced a case of Non-Q myocardial infarction in a 42 years-old female patient with myocardial bridge at the proximal and middle part of left anterior descending coronary artery.
Adult ; Angina Pectoris ; Coronary Vessels ; Female ; Humans ; Milk ; Myocardial Bridging* ; Myocardial Infarction ; Systole

BACKGROUND: Hepatic dysfunction may occur after single, or more usually repeated, exposures to the halogenated inhaled anesthetics. The cause may be free radical snd metabolites of inhaled anesthetics, immune reaction and hypoxic damage by decreasing total flows. The purpose of this study is to evaluate postoperative liver function between the general anesthesia and the thoracic epidural blockade supplemented with the light general anesthesia. METHODS: Thirty patients were divided into two groups; 15 patients were aneshtetized with 2 vo1% of enflurane combined with nitrous oxide(2 I/min) and oxygen(1.5 l/min) in the general anesthesia group, and in 15 patients of the thoracic epidural blockade group thoracic epidural blockade with 2% lidocaine and light general anesthesia using nitrous oxide(2 l/min) and oxygen(1.5 l/min) were performed. Serum glutamic oxalacetic transaminase(SGOT), serum glutamic pyruvic transaminase(SGPT) and alkaline phosphatase were evaluated before anesthesia, 1, 3, 5 and 7 days after gastric surgery on both groups. RESULTS: In the general anesthesia group, postoperative SGOT levels were increased above normal range but were within normal limits in the thoracic epidural blockade group. SGPT level was increased above normal range on postoperative one day in the general anesthesia group but no increase of postoperative SGPT levels was observed in the thoracic epidural blockade group. On postoperative seven days the level of alkaline phosphatase was increased within normal range in the general anesthesia but in the thoracic epidural blockade group the levels of alkaline phosphatase were not increased. CONCLUSIONS: We consider that postoperative liver function is not influenced with thoracic epidural blockade supplemented with light general anesthesia for upper abdominal surgery.
Alanine Transaminase ; Alkaline Phosphatase ; Anesthesia ; Anesthesia, General* ; Anesthetics ; Aspartate Aminotransferases ; Enflurane* ; Humans ; Lidocaine ; Liver* ; Reference Values

BACKGROUND: Development of hematopoietic growth factor made it possible to treat anemia and granulocytopenia following intensive chemotherapy and for thrombocytopenia, recently found thrombopoietin(TPO) is being applied experimentally in several countries. The megakaryocyte colony assay can assess the effect of TPO on the thrombocytopenia resulted from cancer chemotherapy or hematopoietic stem cell transplantation. In vitro colony assay procedures for detecting human erythroid and granulocyte macrophage progenitors have been in widespread use for many years. However, reproducible assay methods for human megakaryocyte progenitors have lagged considerably behind especially in Korea. Duration platelet recovery following transplantation depends on the origin of the hematopoietic cells. Usually thrombocyte recovery is delayed following cord blood stem cell transplantation because of the small amount of cells administered. This study was carried out to investigate and establish the megakaryocyte colony assay of hematopoietic stem cells obtained from the various origin of the hematopoietic stem cells with or without TPO. METHOD: Mononuclear cells of bone marrow, peripheral blood and cord blood were collected following Ficoll density gradient centrifugation and megakaryocyte colony assay was done using MegaCultTM(Stem Cell Tech. Inc., Canada). After liquifying the agarose, mononuclear cells were added and then agarose and cell mixture were dispersed into the two wells of the chamber slide. These slides were incubated for 18~21 days at 37oC, 5% CO2. The megakaryocyte colonies were detected by staining of the cells with a primary antibody to the GPIIb/IIIa antigen, secondary antibody, alkaline phosphatase and Evans Blue in order. Changes of CD34 and GPIIb/IIIa positive cells were also analysed in flask culture using flow cytometry. RESULTS: CD34 positive cells were most abundant in the mononuclear cells of the bone marrow, meanwhile the number of CFU-GM and megakaryocyte colony were greater in the mononuclear cells of the cord blood. After administration of TPO, the cell number of megakaryocyte colony was increased dose dependently, but CFU-GM colony did not show any response to TPO. With flask culture, the cell number was decreased with or without TPO. However adding GM-CSF, IL3 and TPO to cord blood mononuclear cell, the number of the cord blood mononuclear cells was increased on the 5 th day. The amount of CD34 positive cells was increased dose dependently to TPO in one of two cord blood and one peripheral blood. The amount of GPIIb/IIIa positive cells was increased dose dependently to TPO following incubation of all the mononuclear cells. CONCLUSION: This study revealed successful result of megakaryocyte colony assay using MegaCultTM in various kinds of mononuclear cells and suggested that TPO was useful for CFU-mega colony formation. The amount of GPIIb/IIIa positive cells was increased with TPO in the flask culture. Therefore TPO could be useful for assessment of CFU- mega, and could be applied for the in vivo and in vitro expansion of megakaryocytes and platelets.
Agranulocytosis ; Alkaline Phosphatase ; Anemia ; Blood Platelets ; Bone Marrow ; Cell Count ; Centrifugation, Density Gradient ; Cord Blood Stem Cell Transplantation ; Drug Therapy ; Evans Blue ; Fetal Blood ; Ficoll ; Flow Cytometry ; Granulocyte-Macrophage Colony-Stimulating Factor ; Granulocyte-Macrophage Progenitor Cells ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells* ; Humans ; Korea ; Megakaryocyte Progenitor Cells ; Megakaryocytes* ; Sepharose ; Thrombocytopenia

BACKGROUND: The substantia nigra pars reticulata is a critical site for the control of epileptic seizures. The potentiation of the inhibitory gamma-amino butyric acid (GABA) ergic input from the striatum to the substantia nigra pars reticulata suppresses primary or secondary generalized seizures in rats. Recent data suggests that the projection from the subthala-mus to the substantia nigra pars reticulata plays a critical role in the control of some forms of epileptic seizures. METHODS: We examined the possible involvement of the excitatory glutamatergic input from the subthalamic nucleus (STN) to the substantia nigra in pilocarpine-induced seizures by the direct injection of GABA receptor agonist (muscimol) and n-methyl- D-aspartate (NMDA) receptor antagonist (MK-801) into the substantia nigra pars reticularis (SNr) and the subthalamic nucleus respectively. Sprague-Dawley rats were classified into four groups depending on the pretreatment; the injection of saline (STN sham) or muscimol (STN) into the subthalamic nucleus, and the injection of saline (SNr sham) or MK-801 (SNr) into the substantia nigra. Seizure was induced by the intraperitoneal injection of pilocarpine (400 mg/kg). RESULTS: Mean seizure stages in the STN group (1.5 +/-0.5) and SNr group (1.3 +/-0.5) were significantly lower than STN sham group (4.8 +/-0.4) and SNr sham group (4.8 +/-0.4) (P <0.05). Electroencephlograms showed typical status epilepticus patterns in the sham operation group but only discrete ictal discharges with slowing and intermittent ictal spikes were shown in both STN and SNr group. Viable cell numbers in the CA1 and CA3 in STN and SNr groups were significantly higher than those of the sham group (P <0.05). CONCLUSIONS: These results provide evidence for the role of subthalamo-nigral projections in the modulation of pilocarpine-induced seizures.
Animals ; Butyric Acid ; Cell Count ; D-Aspartic Acid ; Dizocilpine Maleate ; Epilepsy ; GABA Agonists ; Injections, Intraperitoneal ; Muscimol ; Pilocarpine* ; Rats* ; Rats, Sprague-Dawley ; Seizures* ; Status Epilepticus ; Substantia Nigra ; Subthalamic Nucleus ; Subthalamus

PURPOSE: The purpose of this study was to determine whether in vitro plain radiography and ultrasonography(US) could predict the outcome of gallstone dissolution with methyl tert-butyl ether(MTBE). METHODS AND MATERIALS: The plain radiography and ultrasonography of 75 gallstones removed at surgery from 75 patients were obtained. The plaih radiographic findings were grouped by their calcification pattern as group l:lucent, group 2:central, group 3:diffuse and group 4: laminated. The ultrasonographic findings were grouped by their echo pattern as group 1 :arc shaped, clearly defined surface echo followed by distinct acoustic shadow, group 2:strong surface echo with gradually attenuating, meniscus shaped inner echo, group 3:strong surface echo with fill-in pattern of internal echo, group 4:strong surface echo with irregular inner echo and group 5:entirely discerning circumsference, homogeneous internal echotexture. After imaging, the gallstones underwent MTBE dissolution for 6 hours. The residual weight of each stone was measured every 2 hours. The correlation between imaging findings and dissolution rate was retrospectively analysed. RESULTS: The radiolucent and central calcification stones were dissolved rapidly as compared with the diffuse and laminated calcification stones(p<.05). There was no statistically significant difference among the groups based on ultrasonographic findings alone. However, after exclusion of the certain calcification groups that were resistent to dissolution-diffuse or laminated calcification stones-all US groups except one that shows wholly circumscribed margin and homogeneous fill-in pattern of internal echo dissolved well to average 20% or less of the original weight(p<.05). CONCLUSION: MTBE dissolution can be tried in stones that meet both ultrasonographic (not a stone with homogeneous fill-in pattern of internal echo) and plain radiographic (radiolucent or cental calcification stone) criteria.
Acoustics ; Ether* ; Gallstones* ; Humans ; Radiography* ; Retrospective Studies ; Ultrasonography*

PURPOSE: Hernia repair after recurrence is a challenging procedure, and many approaches have been suggested for it. Total extraperitoneal (TEP) hernia repair should be considered in recurrent hernia. This study was conducted for the purpose of investigating the clinical usefulness of laparoscopic TEP hernia repair for recurrent inguinal hernia. METHODS: Among the 191 patients who underwent TEP hernia repair at these authors' center from June 2006 to January 2010, the bilateral-hernia cases and the patients with a history of previous pelvic surgery were excluded. A total of 19 patients (12.5%) were enrolled in the recurrent-inguinal-hernia group (group R), and 133 patients (87.5%) in the primary-hernia group (group P). Data were investigated retrospectively, based on the medical records. RESULTS: The mean operation time was 97 minutes in group R and 99 minutes in group P (>0.05). In group R, no operation modality change occurred, and temporary urinary retention was developed in four patients (21.1%). In group P, on the other hand, operation modality change from TEP to the transabdominal preperitoneal approach was necessary in four patients (3%). Additionally, in group P, 30 patients (22.6%) had temporary urinary retention and six (4.5%) had testicular edema. No recurrence was identified during the follow-up period in both groups (mean follow-up period: 15.8 months for group R and 18.0 months for group P). CONCLUSION: Laparoscopic TEP hernia repair seems to be a safe and useful method for correcting recurrent inguinal hernia.
Edema ; Follow-Up Studies ; Hand ; Hernia ; Hernia, Inguinal ; Herniorrhaphy ; Humans ; Laparoscopy ; Pyrazines ; Recurrence ; Retrospective Studies ; Urinary Retention

PURPOSE:The purpose of this study is to evaluate the change of pancreatic size after gastric resection in patients with gastric malignancy. MATERIALS AND METHODS: We compared the pancreatic sizes on pre- and post-operative abdominal CT scans in 50 patients whose stomach had been resected due to malignancy. The mean interval was 20.3 months (range, 5-81 months). The types of the operation were total gastrectomy with esophagojejunostomy and jejunojejunostomy (n=7), and subtotal gastrectomy with gastrojejunostomy (Billroth II) (n=43). Pancreatic thickness was measured perpendicular to the pancreatic long axis at head, body and tail. RESULT: There was a significant reduction of pancreatic size on postoperative CT scan. The reduction rate was 5.9% in the head (p<0.001), 7.1% in the body (p<0.001) and 14.4% in the tail (p<0.001). CONCLUSION:Significant reduction of pancreatic size was observed after gastric resection due to gastric rnalignancy, especially in tail.
Axis, Cervical Vertebra ; Gastrectomy ; Gastric Bypass ; Head ; Humans ; Stomach ; Tomography, X-Ray Computed

To evaluate the usefulness of brain SPECT as a tool with diagnostic and prognostic values m patients with acute cerebral infarction, twenty-four patients who were diagnosed as embolic(n=12) or thrombotic(n7-12) infarction by clinical manifestation and brain CT or MRI were selected. Both 99mTc-HMPAO & 99mTc-DTPA Brain SPECT were performed for each patients within 1 week after the attack Asymmetric index(Al) in both hemispheres was calculated as 200(l(right-left)l/(right+left)). Activities of daily livmg(ADL) was scored by Barthel Index on the admission day and the 21st day after the attack. The relationshiop between ADL index and Al of two types of brain SPECT was analysed by calculating correlation coefficient. 1.In embolic infarction, the Al of 99mTc-DTPA SPECT was markedly higher than that of 99mTc-HMPAO spect, but there was no difference in thrombotic type. 2. The AI of 99m'Tc-DTPA SPECT in embohc infarction was sigiuficantly higher than that m thrombotic type(p valu=.001), but the Al of 99mTc-HMPAO SPECT was similar in both types of infarction. 3.The correlation coefficient Al of 99mTc-DTPA Brain SPECT and ADL m the 21st day after stroke was statistically significated. These results suggested that the 99mTc-DTPA brain SPECT should be useful not only for the differemtial diagnosis between thrombotic and embolic infarctions but also for the prediction of prognosis in patients with ischemic stroke.
Activities of Daily Living ; Brain* ; Cerebral Infarction* ; Diagnosis* ; Humans ; Infarction ; Magnetic Resonance Imaging ; Prognosis* ; Stroke ; Technetium Tc 99m Exametazime ; Technetium Tc 99m Pentetate* ; Tomography, Emission-Computed, Single-Photon*

PURPOSE: The purpose of this study was to evaluate the clinical utility of rapid detection of Down syndrome and Edward syndrome by Interphase Fluorescence in Situ Hybridization (FISH) analysis METHODS: A retrospective study in 309 cases of amniotic fluid samples, analysed by interphase FISH with DNA probes specific to chromosome 18 and 21, was performed. All FISH results were compared with conventional cytogenetic karyotypings. RESULTS: The results were considered as informative and they were obtained within 48 hrs. A case of Down syndrome and a case of Edward syndrome were diagnosed by FISH and confirmed by subsequent cytogenetic analysis. In 12 cases with normal FISH results, the cytogenetic analysis showed a case of partial trisomy 22, three cases of sex chromosomal aneuploidy, two cases of mosaicism, two cases of microdeletion, and four cases of structural rearrangement. CONCLUSION: FISH is a rapid and effective diagnostic method, which can be used as an adjunctive test to cytogenetic analysis, for prenatal identification of chromosome aneuploidies. For the more genome- wide screening with variety of probes, the technique of FISH is both expensive and labor-intensive.