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 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

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*

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: For better understanding of the clinical characteristics and outcomes of management in pediatric urolithiasis, we report our experience with pediatric urolithiasis during the past 10 years. MATERIALS AND METHODS: We retrospectively reviewed the records of 56 pediatric patients with urolithiasis between May 1990 and May 2000. The mean age of the patients was 8.4 years (3 months-18 years) with sex ratio of 1.2:1.0 (male:female). We described initial symptoms, risk factors, location and size of stones, stone composition, treatment outcomes and complications. Metabolic evaluations were performed in 26 patients. RESUTLS: Metabolic abnormalities were found in 13 (23%) and all of them had hypercalciuria. In 3 of these patients, hyperuricosuria was also detected. Urinary tract anomalies were discovered in 10 (18%), all of whom were under age of 10 and underwent surgical reconstruction except for 1 patient who had horseshoe kidney and was lost during follow-up. For the treatment, 28 patients (50%) were treated by SWL. Other treatment modalities consisted of ureteroscopic lithotripsy in 3, pyelolithotomy in 2 who had large staghorn stone (>5cm), and cystolitholapaxy in 2. Spontaneous stone passage was observed in 8 patients (14%). For those who underwent SWL, stone free rates of the first, second and third session were 78%, 96% and 100% respectively. There were no major complications. CONCLUSIONS: Pediatric patients with urolithiasis requires evaluation for metabolic and structural abnormalities. Most of the urinary stone disease in the pediatric age group without structural anomalies could be effectively treated by SWL with minimal morbidity whereas those with structural anomalies necessitating surgical reconstruction are the best candidates for open surgery.
Follow-Up Studies ; Humans ; Hypercalciuria ; Kidney ; Lithotripsy ; Retrospective Studies* ; Risk Factors ; Sex Ratio ; Urinary Calculi ; Urinary Tract ; Urolithiasis*

BACKGROUND AND AIMS: There are two main routes in the growth and development of colo-rectal carcinomas, that is to say, the adenoma-carcinoma sequence and the route described by the de novo theory. The adenoma-carcinoma sequence has been considered a principal route for colorectal carcinomas for a long time, but recently the de novo cancer theory has become a controversial issue, and many authors have claimed that their cases were de novo carcinomas. Since Muto described small "flat elevations" and emphasized their significance as precursors of colorectal carcinomas, especially of flat colorectal carci-nomas, many flat elevations have been reported. He cautioned that the reported de novo carcinomas could also be carcinomas that developed from small flat adenomas. In addition, no data are available in Korea on the subject of flat elevations. Accordingly, we attempted to analyze flat elevations in the colon and to obtain correlations between their endoscopic and histologic features and their clinical implications. METHODS: The authors performed colonoscopic removal of 146 flat elevations from January 1997 to December 1997 at Song-Do Colorectal Hospital in Seoul, Korea. We reviewed and analyzed all those lesions. During colonoscopy, the sizes of the lesions were measured by an endoscopic ruler. RESULTS: The most common age group was the sixth decade. Only one case involved a patient under 30 years old. The male-to-female ratio was 3.2 : 1. The numbers of flat adenomas and flat adenocarcinomas were 68 and 1, respectively. These 69 cases amounted to 47.3% of the flat elevations. The malignancy rate for flat adenomas was 1.4% (1/69). There were no malignancies in flat elevations with diameters below 10 mm. Hyperplasticpolyps and chronic nonspecific inflammation showed 34.9% and 15.8%, respectively. Most of the flat elevations were smaller than 10 mm (98.6%). Recognizable endoscopic features of the flat elevations were redness (47.3%) and depression (15.8%), but 50 cases (34.2%) of the flat elevations had no specific features. Flat elevations smaller than 5 mm had discolorations in 15 cases (14%). The sigmoid colon was the most common site of the flat elevations. The percentage of flat elevations found in the sigmoid colon and the rectum was 70.5%. Hot biopsy was the main treatment for lesions smaller than 5 mm (75.7%), and snare polypectomy was the main treament for lesions from 5 mm to 9 mm. CONCLUSIONS: Flat elevations were detected without difficulty through careful examination. Nearly half of the flat elevations were flat adenomas. Accordingly, flat elevations may have some clinical significance on the basis of the adenoma-carcinoma sequence, but flat elevations greater than 10 mm should be managed cautiously because of possible malignancy.
Adenocarcinoma ; Adenoma ; Adult ; Biopsy ; Colon* ; Colon, Sigmoid ; Colonoscopy ; Colorectal Neoplasms ; Depression ; Growth and Development ; Humans ; Inflammation ; Korea ; Rectum ; Seoul ; SNARE Proteins

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