No abstract available.

No abstract available.
Bronchopulmonary Sequestration* ; Lung* ; Ultrasonography, Prenatal*

No abstract available.
Arrhythmias, Cardiac*

No abstract available.
Asbestos*

PURPOSE:Worldwide incidence of type 2 diabetes mellitus in youth is increased for last 3 decades. We reviewed the clinical characteristics of the patients and their long term complications. METHODS:We reviewed the medical data of 69 patients who were diagnosed type 2 diabetes mellitus at Seoul National University Children's Hospital before the age of 18 years between January 1991 and December 2007. RESULTS:Forty seven patients (68.1%) developed after 2000. The mean age of onset was 13.1+/-2.0 years. Forty seven (69.1%) patients had family history of diabetes mellitus and forty six (66.6%) patients had overweight or obesity. Thirteen (22.4%) patients were small for gestational age but their body mass index wasn't increased compared to that of appropriate for gestational age at the onset of disease. There were 7 patients who had microvascular complication and the mean disease duration was 9.1+/-3.3 years. Hemoglobin A1c was not increased in the complication group compared to the complication-free group. CONCLUSION:The number of childhood and adolescence onset type 2 diabetes mellitus was increased. The proportion of small for gestation age was high and the long term complication developed in adolescence or early adult life. Early intervention of the risk factors and regular evaluation of the complications are needed and systematized follow-up of small for gestational age is also necessary.
Adolescent ; Adult ; Age of Onset ; Body Mass Index ; Diabetes Complications ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Early Intervention (Education) ; Follow-Up Studies ; Gestational Age ; Hemoglobins ; Humans ; Incidence ; Infant ; Obesity ; Overweight ; Pregnancy ; Risk Factors

PURPOSE: Reduced growth and microvascular complications have been recognized as consequences of type 1 diabetes mellitus (T1DM). We assessed the effect of T1DM on growth and factors associated with the development of microvascular complications. METHODS: We conducted a retrospective longitudinal evaluation of 154 patients above 16 years of age. We analyzed factors which affect final height standard deviation scores (SDS) and development of microvascular complications. RESULTS: Final height SDS was -0.11+/-1.15 (-0.26+/-1.33 in females, 0.04+/-0.91 in males). Final height SDS was significantly lower than midparental height SDS and height SDS at diagnosis. There was no difference in final height SDS according to age at onset, existence or nonexistence of complications, or average HbA1C. Height SDS at onset of puberty, midparental height SDS and pubertal growth gain affected final height SDS. The number of patients with complications was 37 (24 percent). Microvascular complications developed at a younger age and after longer duration of diabetes in patients with a prepubertal onset of T1DM compared to patients with pubertal onset. Patients with complications had a higher level of average HbA1C than patients without complications. Patients whose microalbuminuria regressed had lower levels of average HbA1C, systolic BP, second 24h urine microalbumin than patients with persistant or progressed microalbuminuria. CONCLUSION: The results suggest that degrees of glycemic control don't affect final height, but various factors associated with T1DM can impair growth potential. Additionally, the degrees of glycemic control and puberty affect the development of microvascular complications.
Adolescent ; Diabetes Mellitus, Type 1* ; Diagnosis ; Female ; Humans ; Puberty ; Retrospective Studies

Synovial sarcoma occurring in the pleura and lung is extremely rare. We report a case of pleuropulmonary synovial sarcoma as a second malignant neoplasm. The patient had been diagnosed with acute myelomonocytic leukemia at 5 years of age, and received matched sibling donor allogeneic bone marrow transplantation, with total body irradiation and cyclophosphamide conditioning. At 22 years of age, he complained of worsening chest discomfort and exertional dyspnea. Chest CT revealed a huge mass in the right middle lobe, pleura, and diaphragm. The patient was initially diagnosed as sarcomatoid malignant mesothelioma, without any environmental or occupational asbestos exposure. Five months later, the patient presented with soft tissue metastasis and underwent needle biopsy. Pathological examination including SYT-SSX RT-PCR revealed synovial sarcoma, which led to a review of the original tumor findings and confirmed the diagnosis of pleuropulmonary synovial sarcoma.To the best of our knowledge, our patient is the first case of pleuropulmonary synovial sarcoma developed after allogeneic hematopoietic stem cell transplantation.

Synovial sarcoma occurring in the pleura and lung is extremely rare. We report a case of pleuropulmonary synovial sarcoma as a second malignant neoplasm. The patient had been diagnosed with acute myelomonocytic leukemia at 5 years of age, and received matched sibling donor allogeneic bone marrow transplantation, with total body irradiation and cyclophosphamide conditioning. At 22 years of age, he complained of worsening chest discomfort and exertional dyspnea. Chest CT revealed a huge mass in the right middle lobe, pleura, and diaphragm. The patient was initially diagnosed as sarcomatoid malignant mesothelioma, without any environmental or occupational asbestos exposure. Five months later, the patient presented with soft tissue metastasis and underwent needle biopsy. Pathological examination including SYT-SSX RT-PCR revealed synovial sarcoma, which led to a review of the original tumor findings and confirmed the diagnosis of pleuropulmonary synovial sarcoma.To the best of our knowledge, our patient is the first case of pleuropulmonary synovial sarcoma developed after allogeneic hematopoietic stem cell transplantation.

OBJECTIVE: We assessed the outcomes of a simplified technique for the percutaneous placement of a hepatic artery port-catheter system for chemotherapy infusion in advanced hepatocellular carcinoma with portal vein invasion. MATERIALS AND METHODS: From February 2003 to February 2008, percutaneous hepatic artery port-catheter insertion was performed in 122 patients who had hepatocellular carcinoma with portal vein invasion. The arterial access route was the common femoral artery. The tip of the catheter was wedged into the right gastroepiploic artery without an additional fixation device. A side hole was positioned at the distal common hepatic artery to allow the delivery of chemotherapeutic agents into the hepatic arteries. Coil embolization was performed only to redistribute to the hepatic arteries or to prevent the inadvertent delivery of chemotherapeutic agents into extrahepatic arteries. The port chamber was created at either the supra-inguinal or infra-inguinal region. RESULTS: Technical success was achieved in all patients. Proper positioning of the side hole was checked before each scheduled chemotherapy session by port angiography. Catheter-related complications occurred in 19 patients (16%). Revision was achieved in 15 of 18 patients (83%). CONCLUSION: This simplified method demonstrates excellent technical feasibility, an acceptable range of complications, and is hence recommended for the management of advanced hepatocellular carcinoma with portal vein thrombosis.
Angiography ; Antineoplastic Agents/*administration & dosage ; Carcinoma, Hepatocellular/*drug therapy/pathology ; *Catheters, Indwelling/adverse effects ; Embolization, Therapeutic ; Female ; Femoral Artery/surgery ; Fluoroscopy ; *Hepatic Artery ; Humans ; Infusions, Intra-Arterial ; Liver Neoplasms/*drug therapy/pathology ; Male ; Middle Aged ; Portal Vein/*pathology ; *Radiography, Interventional ; Treatment Outcome

PURPOSE: The purpose of this study was to evaluate the effect of amino acid mixtures on incidence and severity of total parenteral nutrition associated-cholestasis(PNAC) in very low birth weight infants. METHODS: Retrospective review of 63 very low birth weight infants(birth weight < or =1,500 g) who received total parenteral nutrition(TPN) in our neonatal intensive care unit from January 2000 to December 2004 was performed. Patients were divided into 2 groups : Group I(n=32, Jan 2000-Jun 2002) and Group II(n=31, Jul 2002-Dec 2004), where infants in Group II received taurine and glutamic acid-rich amino acid mixtures. PNAC was defined as serum direct bilirubin(DB) level greater than 2.0 mg/dL. The incidence and severity of PNAC were compared between these groups. RESULTS: The incidence of PNAC was significantly lower in Group II than in Group I(21.9% vs 6.5%, P<0.148). Maximum and mean DB levels were also significantly lower in Group II(P<0.05). CONCLUSION: The incidence and severity of PNAC in very low birth weight infants may be reduced with different composition of amino acid mixtures in TPN. Further prospective randomized controlled studies are needed to determine an ideal composition of acid mixtures to prevent the development of PNAC.
Cholestasis* ; Humans ; Incidence ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Intensive Care, Neonatal ; Parenteral Nutrition, Total ; Retrospective Studies ; Taurine