BACKGROUND: Immune thrombocytopenic purpura (ITP) is a frequently observed bleeding disorder in children. High dose intravenous immunoglobulin G (IVIG) has been used for the treatment of ITP since 1981, and now several methods of IVIG infusion are used. Since 1983, we have treated ITP patients with short-term and low-dose IVIG according to the individual patient's daily response. This study aimed to evaluate individual patient's response after IVIG for the prediction of chronic ITP.METHODS: We evaluated 259 childhood ITP patients retrospectively who were newly diagnosed at the Department of Pediatrics, Kyungpook National University Hospital from 1983 to 2012. We analyzed the individual response to treatment and current state of disease. We evaluated the time to reach desired platelet counts after treatment of IVIG, relapse rate and diagnosis of chronic ITP. The patients were classified into 2 groups according to the time to reach desired platelet counts (50,000/microL) after daily treatment of IVIG, rapid (1 or 2 doses) and slow responder (more than 3 doses).RESULTS: Among 182 patients followed up over 6 months, 41 patients (22.5%) were eventually diagnosed with chronic ITP. Hundred and two patients (56.7%) belonged to rapid response group, and 17 of them (16.7%) were diagnosed with chronic ITP. Eighty patients (44.4%) belonged to the slow response group, and 24 of them (30%) were diagnosed with chronic ITP, which were higher than the early response group (P=0.033).CONCLUSION: Individual response rate of IVIG treatment could be a useful predictor of chronic ITP, but this finding needs support from further studies.
Child ; Diagnosis ; Hemorrhage ; Humans ; Immunoglobulin G ; Immunoglobulins ; Immunoglobulins, Intravenous ; Methods ; Pediatrics ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic ; Recurrence ; Retrospective Studies

BACKGROUND: The immune thrombocytopenia (ITP) criteria were newly standardized by the International Working Group. Thus, we analyzed the natural course of childhood chronic ITP to predict the prognosis based on the revised criteria. METHODS: The medical records of children with chronic ITP from May 2000 to February 2013 in our institute were reviewed. RESULTS: Forty-seven children with chronic ITP who were not undergoing corticosteroid therapy were included. Their initial platelet count was 23+/-25x10(9)/L, and age at diagnosis was 6.3+/-4.1 years. The follow-up period was 5.4+/-3.7 years. Among them, 44.7% (21/47) showed spontaneous remission and maintained a platelet count > or =100x10(9)/L. And 66.0% (31/47) maintained a platelet count > or =50x10(9)/L until the last follow-up date. The time periods required for the platelet count to be maintained > or =50x10(9)/L and > or =100 x10(9)/L were 3.1+/-2.7 and 3.6+/-2.7 years. Age at diagnosis in the > or =50x10(9)/L group (5.7+/-4.4 years) was significantly lower than the age at diagnosis in the <50x10(9)/L group (7.4+/-3.3 years) (P=0.040). And follow-up period was the factor influencing prognosis between the > or =100x10(9)/L group and <50x10(9)/L group (P=0.022). CONCLUSION: Approximately 45% of children with chronic ITP recovered spontaneously about 3-4 years after the diagnosis and 2/3 of patients maintained a platelet count > or =50x10(9)/L, relatively safe state. Age at diagnosis of ITP and follow-up period were the factors influencing prognosis in this study.
Child ; Diagnosis ; Follow-Up Studies ; Humans ; Medical Records ; Platelet Count ; Prognosis ; Remission, Spontaneous ; Thrombocytopenia*

BACKGROUND: The effect of arterial carbon dioxide tension (PaCO2) during ischemia and reperfusion has been a controversial issue. In this study, the effect of PaCO2 during ischemia and reperfusion was evaluated by 31P magnetic resonance spectroscopy (MRS). METHODS: Incomplete global cerebral ischemia was induced by ligation of carotid artery under lowered mean blood pressure (mean blood pressure= 40 mmHg) for 30 minutes followed by 2 hours of reperfusion. Eighteen cats were divided into 3 groups: For group 1 (n=6) (control group), animals were subjected to normocarbia (PaCO2=28~33 mmHg) during ischemia and reperfusion, for group 2 (n=6), animals were subjected to hypocarbia (PaCO2=18~23 mmHg) during ischemia and reperfusion, and for group 3 (n=6), animals were subjected to normocarbia during ischemia and hypocarbia during reperfusion. RESULTS: For group 1, the energy metabolism measured by [PCr/Pi] was recovered about 74.7 6.4%. For group 2, the energy metabolism failed to be completely recovered by 120 minutes of reperfusion (69.3 7.3%), whereas for group 3, the energy matabolism was completely recovered by 120 minutes of reperfusion (97.6 2.4%). There were statistically significant differences between group 1 and group 3 (p<0.05). The changes in pH were not significantly different among the groups. CONCLUSION: In this study, a condition of hypocarbia during reperfusion seems better for the energy metabolism after incomplete global ischemia of cats.
Animals ; Blood Pressure ; Brain Ischemia ; Brain* ; Carbon Dioxide ; Carotid Arteries ; Cats* ; Energy Metabolism* ; Hydrogen-Ion Concentration ; Ischemia ; Ligation ; Magnetic Resonance Spectroscopy ; Reperfusion

Neurological complications occur frequently in patients undergoing liver transplantation. We report a case of multi-ple neurologic complications including central pontine and extrapontine myelinolysis, seizure, and mutism after liver transplantation. Though the cause would be obscure and multifactorial, the hyponatremia with its rapid correction dur-ing operation and the side effects of the immunosuppressant would be the most probable causes in this case.
Humans ; Hyponatremia ; Liver Transplantation* ; Liver* ; Mutism ; Myelinolysis, Central Pontine ; Seizures

Systemic lupus erythematosus (SLE) which is thought to be autoimmune in nature affects multiple organs and produces a diversity of signs and symptoms. However, cardiovascular manifestations of SLE are manifested more frequently by autopsy. Recently, with the prolonged survival and improvement of diagnostic methods in SLE including echocardiography, the morbidity and mortality associated with cardiovascular manifestations of SLE became more apparent and increased. Simultaneous involvement of the pulmonary artery and the myopericardium in SLE is known to be rare. Pulmonary hypertension is known to be associated with poor prognosis. We report a 27 year-old female patient of SLE with pulmonary hypertension, pericarditis and left ventricular systolic dysfunction.
Adult ; Autopsy ; Echocardiography ; Female ; Humans ; Hypertension, Pulmonary* ; Lupus Erythematosus, Systemic* ; Mortality ; Pericarditis* ; Prognosis ; Pulmonary Artery

PURPOSE: 45,XY,t(13;14)(q10;q10) karyotype can suggest infertility associated with more or less severe oligospermia in male adults. In addition, 45,XX,t(13;14)(q10;q10) karyotype carries reproductive risks such as miscarriage or infertility in female adults. However, reports on the phenotype of this karyotype in children are very rare. This study was done to observe various phenotypes of this karyotype in children. METHODS: Between January 2007 and December 2012, children diagnosed with 45,XY,t(13;14)(q10;q10) or 45,XX,t(13;14)(q10;q10) karyotype by chromosome analysis were analyzed retrospectively. RESULTS: Eight children (5 boys and 3 girls) were diagnosed with 45,XY,t(13;14)(q10;q10) or 45,XX,t(13;14)(q10;q10) karyotype. They ranged in age from 5 years and 6 months to 12 years and 4 months. The phenotypes of the study patients consisted of 1 hypogonadotrophic hypogonadism, 1 precocious puberty, 3 early puberty, 2 growth hormone deficiency (GHD) (partial) and 1 idiopathic short stature. As shown here t(13;14)(q10;q10) Robertsonian translocation shows a wide range of phenotypes. CONCLUSION: It can be said that t(13;14)(q10;q10) Robertsonian translocation shows various phenotypes from GHD to precocious puberty in children. Further large-scale studies are necessary.
Child* ; Endocrine System Diseases* ; Female ; Growth Hormone ; Humans ; Hypogonadism ; Karyotype ; Male ; Phenotype ; Puberty ; Puberty, Precocious ; Translocation, Genetic*

Leimyosarcoma of the inferior vena cava is a rare disorder and may present with symptoms of obstruction of the normal flow of blood. We report a case of leiomyosarcoma of the inferior vena cava in 55-year-old female patient. The tumor was discovered incidentally by ultrasonography of the abdomen which was performed to evaluate epigastric pain and indigestion. Further radiological studies including CT and inferior vena cavography confirmed the presence of a 5cm sized mass in the inferior vena cava . The microscopic examination of a specimen obtained by a catheter from inferior vena cava mass revealed malignant mesenchymal tumor. The tumor was completely resected with reconstruction of the inferior vena cava and left renal vein. The diagnosis of leiomyosarcoma was made by micoscopic and immunohistochemical findings of the resected tumor.
Abdomen ; Catheters ; Diagnosis ; Dyspepsia ; Female ; Humans ; Leiomyosarcoma* ; Middle Aged ; Renal Veins ; Ultrasonography ; Vena Cava, Inferior*

PURPOSE: The majority of patients with early gastric cancer show long-term survival after surgery. So a special attention must be directed to preserving gastric function in these patients. When node-negative early gastric cancer could be diagnosed preoperatively, then minimally invasive surgery can be performed to ensure a postoperative better quality of life. MATERIALS AND METHODS: The pathological records of 2,137 consecutive patients with early gastric cancer who underwent curative operations from January 1986 to December 1998 at Seoul National University Hospital were reviewed. RESULTS: Lymph node metastases were observed in 285 patients (13.3%). In mucosal carcinoma, lymph node metastases were observed in 50 of 1,108 cases (4.5%), and in submucosal carcinoma, in 234 of 1,026 cases (22.8%). The tumor size, depth of invasion and gross appearance were associated with lymph node metastasis. In mucosal carcinoma, the size and histologic differ entiation were associated with lymph node metastasis. In submucosal carcinoma, the size and gross appearance were associated with lymph node metastasis. CONCLUSION: In early gastric cancer, the limited surgery can be applied only to cases satisfying the following criteria; (1) mucosal tumor, (2) size < or =2 cm, (3) elevated type or (4) depressed type which are histologically differentiated and (5) size < or =1 cm among the depressed type his tologically undifferentiated.
Humans ; Lymph Nodes* ; Neoplasm Metastasis* ; Quality of Life ; Seoul ; Stomach Neoplasms* ; Surgical Procedures, Minimally Invasive

PURPOSE: In the fifth edition of International Union Against Cancer (UICC) TNM, nodal staging for gastric cancer is based on the number of metastatic lymph nodes. Variability in the extent of lymphadenectomy and lymph node retrieval can affect the number of metastatic lymph nodes. In this study, the authors attempted to evaluate the influence of nodal yields on the staging of gastric cancer and survival rates. METHODS: A retrospective study was performed in 4354 consecutive patients with gastric cancer, who had undergone curative resection (R0) with nodal yields of 15 or more from 1986 to 1995. Patients were classified into three groups according to the number of nodes examined: patients with nodal yields of 15 or more but less than 30 for group A, 30-39 for group B, and 40 or more for group C. The number of metastatic lymph nodes and the survival rates for each pTNM stage were analyzed for each group. RESULTS: The number of metastatic lymph nodes significantly increased with nodal yields. Greater nodal yields resulted in a higher survival rates with a statistically significant difference between patients with nodal yields of 30 or more, and those with less than 30 in stage IB (p<0.05) and IIIB (p<0.01). CONCLUSION: Our results suggest two possibilities of stage migration and survival benefit according to the difference of nodal yields. Therefore, for minimizing stage migration and maximizing the benefit of survival, at least 30 or more lymphnodes should be resected and examined in gastric cancer surgery.
Humans ; Lymph Node Excision ; Lymph Nodes ; Retrospective Studies ; Stomach Neoplasms* ; Survival Rate

BACKGROUND: Laryngoscopy and tracheal intubation often provoke an undesirable increase in blood pressure and heart rate. This study was done to determine median effective dose (ED50) of nicardipine for prevention of hemodynamic response to tracheal intubation during induction of anesthesia with thiopental, propofol, or etomidate. METHODS: Fourty-five ASA physical status 1 adult patients were allocated into three group; thiopental group (n = 15), propofol group (n = 15), and etomidate group (n = 15). The first patient of each groups received 10microgram/kg of nicardipine 1 minute before induction. Subsequent dose was determined by the hemodynamic response of the previous patient to tracheal intubation based on Dixon's up and down sequential allocation. The test dosing interval was set at 3microgram/kg. If mean arterial pressure increased more than 20% after tracheal intubation, dose of the subsequent patient was increased by 3microgram/kg. If not, it was decreased by 3microgram/kg. Blood pressure was measured after arrival at the operating room, before tracheal intubation, and 1, 2, 3, 4, and 5 minutes following intubation by non invasive method. RESULTS: ED50 of nicardipine for attenuation of hypertensive response after tracheal intubation were 18.0microgram/kg (95% Confidence Limit [CL], 14.8-22.0microgram/kg), 6.2microgram/kg (CL, 2.6-9.5microgram/kg), and 16.7microgram/kg (CL, 13.6-20.7microgram/kg) in thiopental group, propofol group andetomidate group, respectively. CONCLUSIONS: We concluded that less nicardipine dose in propofol group was needed to prevent hypertensive response after tracheal intubation (P < 0.05).
Adult ; Anesthesia ; Arterial Pressure ; Blood Pressure ; Etomidate* ; Heart Rate ; Hemodynamics ; Humans ; Intubation* ; Laryngoscopy ; Nicardipine* ; Operating Rooms ; Propofol* ; Thiopental*