Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result could prove to be unfortunately fatal. The mechanism which underlies the remote damaging effect of neural crest tumor, especially neuroblastoma, on the nervous system resulting in myoclonic encephalopathy is by no means clear. In addition the nature and the extent of the pathologic lesion are inconsistent. We experienced a case of myoclonic encephalopathy associated with an occult mediastinal ganglioneuroblastoma in a 22-month-old girl who was hospitalized for inability to walk without support and tilting of the head to the left side. She became increasingly ataxic, and during the hospitalization myoclonic jerks of upper extremities and head along with chaotic, rapidly flickering, multidirectional spontaneous eye movements, were noted. Laboratory data included normal complete blood count, urinalysis, BUN and creatinine, electrolytes and bone marrow. Chest X-ray and chest CT revealed a relatively well marginated right posterior mediastinal mass. In a 24 hours urine excretion test, VMA and catecholamines were increased. Over the next 2 weeks, a surgical exploration revealed a right posterior mediastinal mass. Microscopically the mass proved to be a ganglioneuroblastoma, extending to right innominate artery and right axillary lymph nodes. Within 2 weeks after the surgery, radiotherapy (2,400 rads) and chemotherapy (CTX, DTIC, VCR) were started, but corticosteroid was not used. She has been free of tumor and abnormal neurological systemic symptoms and signs for 1 1/2 year since the completion of chemotherapy. In the 3 1/2 years follow-up period, her neurologic symptoms has completely resolved by the completion of 2 years chemotherapy. We report a case of mycoclonic encephalopathy associated with hidden ganglioneuroblastoma in 22-month-old girl.
Blood Cell Count ; Bone Marrow ; Brachiocephalic Trunk ; Catecholamines ; Creatinine ; Dacarbazine ; Diagnosis ; Drug Therapy ; Electrolytes ; Epilepsies, Myoclonic* ; Eye Movements ; Female ; Follow-Up Studies ; Ganglioneuroblastoma* ; Head ; Hospitalization ; Humans ; Infant ; Lymph Nodes ; Myoclonus ; Nervous System ; Neural Crest ; Neuroblastoma ; Neurologic Manifestations ; Paraneoplastic Syndromes* ; Radiotherapy ; Thorax ; Tomography, X-Ray Computed ; Upper Extremity ; Urinalysis

Lovastatin(Mevacor) belongs to a new generation of potent drugs for the treatment of hypercho- lesterolemia. It acts by competitive inhibition of 3-hydroxyl-3-methylglutaryl coenzyme A(HMG-CoA) reductase, diminishing in that way the cholesterol synthesis in the liver. HMG-CoA reductase inhibitors also affect cutaneous lipid content. This can induce cutaneous side effects such as eczernatous rashes or xerotic, ichthyosiform lesions due to abnormality in the barrier function in some patients. We report a 54-year-old male patient who developed ichthyosiform skin lesions under treatment with lovastatin(Mevacor) for hypercholesterolemia.
Cholesterol ; Exanthema ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Hypercholesterolemia ; Liver ; Lovastatin ; Male ; Middle Aged ; Oxidoreductases ; Skin*

No abstract available.
Burns* ; Congenital Abnormalities* ; Free Tissue Flaps*

No abstract available.
Hypopituitarism* ; Magnetic Resonance Imaging ; Pituitary Gland*

The association of colonic atresia in patients with Down syndrome is a rare anomaly. The incidence of congenital atresia of the gastrointestinal tract has been estimated to be about one in 1500 births. Colonic atresia is rarer still, and is throut to comprise about 5% to 10% of this group. This intestinal atresia occurs in about 30% to 50% of patient with Down syndrome. We experienced a case of Down syndrome associated with colonic atresia in a 1 day old male. His initial chief complaints at the admission were severe abdominal distension and Down appearance. Diagnosis was confirmed by chromosomal study and operative laparotomy with end-to-end ileodescending colostomy. We report the case with brief review of related literatures.
Colon* ; Colostomy ; Diagnosis ; Down Syndrome* ; Gastrointestinal Tract ; Humans ; Incidence ; Intestinal Atresia ; Laparotomy ; Male ; Parturition

No abstract available.

No abstract available.
Epinephrine* ; Heart Arrest*

BACKGROUND: Video-assisted thoracic surgical procedure via thoracoscopy has recently gained popularity, as it avoids a thoracotomy, reducing intraoperative blood loss, postoperative pain, respiratory dysfunction and hospital stay. However, to visualize adequately the intrathoracic structures, creation of artificial pneumothorax by carbon dioxide insufflation during thoracoscopy would cause significant hemodynamic compromise. The aim of this study was to evaluate the effect of CO2 insufflation into the pleural cavity on the hemodynamics and the arterial blood gas tension under general anesthesia. METHODS: Twenty-five patients, after intubation with single lumen endotracheal tube, underwent enflurane (1~2%) and N2O-O2 (1:1) general anesthesia. Before placement of a thoracoscope, the baseline mean arterial pressure and heart rate were obtained. Measurements were taken at 5, 10, and 20 min. after the beginning of carbon dioxide insufflation (3~5 mmHg) and 10 min. after gas evacuation. Blood gas analyses were done before, during CO2 insufflation and after CO2 evacuation. Data were analyzed using Student t-test. RESULTS: Positive-pressure CO2 insufflation (3~5 mmHg) caused a decrease of mean arterial pressure (5~7%) and an increase of airway pressure (1.5 times) and heart rate (13~20%) throughout the gas insufflation period (p<0.05). Blood gas analyses revealed no significant change. CONCLUSIONS: These results suggest that low CO2 insufflation pressures (3~5 mmHg) may cause cardiovascular depression during thoracoscopy. Therefore careful monitorings should be done during this procedure.
Anesthesia, General* ; Arterial Pressure ; Blood Gas Analysis ; Carbon Dioxide* ; Carbon* ; Depression ; Enflurane ; Heart Rate ; Hemodynamics* ; Humans ; Insufflation* ; Intubation ; Length of Stay ; Pleural Cavity ; Pneumothorax, Artificial ; Postoperative Hemorrhage ; Thoracic Surgical Procedures ; Thoracoscopes ; Thoracoscopy* ; Thoracotomy

PURPOSE: To assess the results of staged MIPO (Minimally Invasive Plate Osteosynthesis) for proximal tibial fractures with compromised soft tissue. MATERIALS AND METHODS: Eighteen proximal tibial fractures (AO 41:9 cases, AO 42:9 cases) included this study. Ten were open fractures. After temporary external fixation until soft tissue healed (mean 27.3 days), MIPO was performed secondarily without bone graft. We assessed the bony union and knee function, and affecting factors of the results were investigated. RESULTS: All fractures united at 20 weeks (range, 11~32) except 1 case. Mean range of knee flexion was 134.4degrees and mean IOWA knee score was 89.1. There were 2 superficial and 2 delayed deep infections from open fractures (grade II:1 case, grade III:3 cases), although they healed after implant removal. Open fractures seem to influence the infection rate. Otherwise, there was no related factor affecting the results. CONCLUSION: MIPO after temporary external fixation can provide favorable results in proximal tibial fractures with soft tissue injuries, but attention of delayed infection should be paid in open fractures.
Fractures, Open ; Iowa ; Knee ; Soft Tissue Injuries ; Tibial Fractures ; Transplants

The kidney size is of special interest during the diagnostic work up of acute lymphocytic leukemia. But is still uncertain whether a finding of kidney enlargement at presentation has long-term prognostic value. We therefore reviewed the kidney size in children with acute lymphocytic leukemia by abdominal ultrasonograms at the time of diagnosis (n=54) and exmined if there was any statistical significance between the kidney size and prognostic parameters and later outcome. The following results were obtained 1) Among the patients whose kidney size was enlarged over 4SD from the normal, hepatomegaly in 3 cases (20%), splenomegaly in 3 cases (20%), mediastinal widening in 2 cases (13%) were noted. The interrelation between kidney size and infiltration of extramedullary system had no statistical significance. 2) Among the patients whose kidney size was enlarged over 4SD from the normal, patients under age 2 and over age 10 in 5 cases (33%), male in 8 cases (53%), involvement of central nervous system in 1 cases (7%), WBC count over 100x10E9/L in 3 cases (20%), Hb over 10 g/dl in 3 cases (20%) and platelet count below 100x10E9/L in 3 cases (20%) were noted. There was no statistical significance between kidney size and infiltration of extramedullary system. 3) Two Year survival rate based upon kidney size was; 95% in the group below 2SD, 79% between 2SD and 4SD, 59% over 4SD. And 2 Year event free survival rate was 71%, 56% and 58 respectively. In conclusion, the kidney size in childhood acute lymphocytic leukemia at the time of diagnosis influences the late outcome, but it is not a meaningful prognostic parameters.
Central Nervous System ; Child ; Diagnosis ; Disease-Free Survival ; Hepatomegaly ; Humans ; Kidney ; Male ; Platelet Count ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Splenomegaly ; Survival Rate ; Ultrasonography