No abstract available.
Gliosarcoma*

Pseudohypoaldosteronism type 1 is a genetic renal tubular disease of salt wasting, presenting in young infants. Tubular unresponsiveness to elevated endogenous and exogenous aldosterone is the suggested pathogenetic mechanism. Oral sodium chloride supplementation relieve the clinical symptoms and electrolyte distrubances. We experienced 2 cases of PHA type 1 in 38-day and 45-day old male infants who were presented with failure to thrive, vomiting and/or dehydration. Laboratory data showed hyponatremia, hyperkalemia, hypochloremia and metabolic acidosis. Renal and adrenal functions were normal. Plasma renin activity and plasma aldosterone concentration were markedly elevated. Under the diagnosis of pseudohypoaldosteronism type 1, oral supplementation of NaCl and/or kayexalate improved the clinical states of the patients.
Acidosis ; Aldosterone ; Dehydration ; Diagnosis ; Failure to Thrive ; Humans ; Hyperkalemia ; Hyponatremia ; Infant ; Male ; Plasma ; Pseudohypoaldosteronism* ; Renin ; Sodium Chloride ; Vomiting

No abstract available.
Intussusception* ; Ultrasonography*

No abstract available.
Coronary Artery Disease* ; Coronary Vessels* ; Dobutamine* ; Echocardiography, Stress* ; Humans ; Myocardium* ; Tomography, Emission-Computed, Single-Photon*

BACKGROUND: To compare the diagnostic usefulness of dobutamine stress echocardiography(DSE) and 99mTc-methoxyisobutyl isonitrile SPECT (MIBI SPECT), two studies were performed simultaneously. METHOD: Fifty-six consecutive patients undergoing coronary angiogram and MIBI SPECT for clinical indications without clincal evidence of myocardial infarction were studied prospectively. During the DSE, MIBI was injected at peak stress, and post-stress images of MIBI SPECT were required on hour later. Both echocardiographic and MIBI SPECT images were visually analysed in a blind fashion. RESULTS: On the basis of coronary angiographic findings, the sensitivites of the DSE and MIBI SPECT (n=36) were 89% and 86%, respectively. The specificities of those (n=20) were 90% and 85%, respectively. Among 33 patients without resting perfusion defect on MIBI SPECT, resting regional wall motion abnormalities on DSE were found in only one patient, whereas, resting perfusion defect on MIBI SPECT were found in 9 patients among 41 patients without resting regional wall motion abnormalities on DSE. Among 17 patients who had resting perfusion defects with partial reversibility on MIBI SPECT, resting wall motion abnormalities were present in 11 patients and five of them showed improvement in the regional wall motion during low dose dobutamine infusion. CONCLUSION: Both dobutamine stress echocardiography and MIBI SPECT are useful methods in the detection of the coronary artery disease, however, MIBI SPECT seems to overestimate the regional ischemic myocardium with contractile reserve that can hardly be evaluated with MIBI SPECT.
Coronary Artery Disease* ; Coronary Vessels* ; Dobutamine* ; Echocardiography ; Echocardiography, Stress* ; Humans ; Myocardial Infarction ; Myocardium* ; Perfusion ; Prospective Studies ; Tomography, Emission-Computed, Single-Photon*

No abstract available.
Arteries* ; Humans ; Intracranial Aneurysm* ; Single Parent*

We describe a case of fine needle aspiration cytology of metastatic nasopharyngeal carcinoma in the lymph node of the neck presenting a predominantly spindle cell pattern. A 36 year-old male patient complained of dysesthesia on the right face and a palpable neck mass. Fine needle aspiration was done on the neck mass. Tumor cells were present in syncytial groups or singly with mainly spindle shaped nuclei, vesicular chromatin, thin and regular nuclear membrane, occasional prominent nucleoli and a few foci of cellular cohesiveness. The cytoplasm was scant and pale with ill-defined borders. Mature lymphocytes were present in the background of aspirates and within the tumor cell clusters. Histologically, the tumor of nasopharynx showed several areas of spindle cell pattern. Because the tumor cells showed a predominantly spindle shape with vesicular nuclear chromatin, the differential diagnosis of spindle cell sarcoma or granuloma of epithelioid cells were considered, but the characteristic morphology of the nuclei with vesicular chromatin and prominent nucleoli, and cellular cohesiveness were important in making the diagnosis of nasopharyngeal carcinoma. The possibility of metastatic carcinoma should always be considered in fine needle aspiration cytology of the lymph node in the neck because the incidence of metastatic carcinoma, particularly of the nasopharyngeal carcinoma in the lymph nodes of the neck is relatively high.
Child ; Male ; Female ; Humans ; Diagnosis, Differential ; Incidence ; Neoplasm Metastasis

We present a family of FSP with review of literatures. Seeligmuller (1876) and Strumpell (1880) were the first to describe familial case of spastic paraplegia characterized by progressive weakness and spasticity of the lower limbs with little or no involvement of the upper extremities. This syndrome is heterogenous in inheritance, age of onset, severity and associated signs. A pure type without complications has been named "Strumpell's FSP". We have recently experienced a case of 17 years old male who had bilateral weakness and stiffness of lower extremities, and gait disturbance with family history. Differential diagnosis, particularly with familial amyotrophic lateral sclerosis, is discussed.
Adolescent ; Age of Onset ; Amyotrophic Lateral Sclerosis ; Diagnosis, Differential ; Gait ; Humans ; Lower Extremity ; Male ; Muscle Spasticity* ; Paraplegia* ; Upper Extremity ; Wills

Primary malignancy of the thymus gland in myasthenia gravis remains rare, especially metastasis of the malignancy to a distant organ. We have presented a case of myasthenia gravis with invasive thymoma and its meatastasis to the diaphragm. The literature concerned with this aspect is reviewed.
Diaphragm ; Humans ; Myasthenia Gravis ; Neoplasm Metastasis* ; Thymoma* ; Thymus Gland

PURPOSE: The Pediatric Risk of Mortality(PRISM) III score was developed from the Physiologic Stability Index(PSI) to assess pediatric ICU mortality and Provide an objective data as a severity index. Although the PRISM score has been applied to many comparisions and analyses in previous studies, there are few reports applied to pediatric intensive care patients in Korea. To evaluate the effectiveness of the PRISM III score as a severity index for expecting mortality and find important variables influencing mortality, we applied this scoring scale to pediatric neurologic patients admitted to the ICU and analyzed the data statistically. METHODS: Data collection was done by careful review of medical records and scored each clinical variable. The outcome at discharge was determined as non-survival, survival, and hopeless discharge. Determination of mortality in the hopeless discharge group was done within 48 hours after discharge by telephone interview. The study populations were classified into four groups; CNS infection(26 patients), acute encephalopathy(31 patients), status epilepticus(35 patients) and cerebrovascular disorder(4 patients). The difference of the PRISM III score between the survival group and non-survival group was compared by using the nonparametric Mann~Whitney test in the entire study population and for each diagnostic group. To confirm the degree of fitness between the actual mortality and Predicted mortality, the Hosmer-Lemeshow goodness-of-fit test, a multiple logistic regression model was used. All clinical variables used for scoring were compared for survivals and non-survivals by the Chi-square test. f values <0.05 were considered significant. RESULTS: The PRISM III score was significantly higher in the non-survival groups than in the survival group. Predicted mortality from the PRISM III score has fitted to actual mortality According to the results of analyses in each diagnostic groups, the PRISM III score was higher in non-survivals of the acute encephalopathy and CNS infection groups, but statistically insignificant in the cerebrovascular disorders and status epilepticus groups. The important variables of the PRISM III score associated with mortality were mental state, Pupil reflex, systolic blood pressure, acidosis, blood sodium level blood creatinine level, blood glucose level, and PT/PTT. , CONCLUSION: The PRISM III score is helpful in predicting mortality in pediatric intensive care neurologic patients, especially those in the acute encephalopathy or the CNS infection groups. However, this score was not useful in the status epilepticus group, and insignificant in cerebrovascular group. Due to the smallness of the study group, more massive and comprehensive studies are needed as a follow up to this study.
Acidosis ; Blood Glucose ; Blood Pressure ; Cerebrovascular Disorders ; Creatinine ; Data Collection ; Follow-Up Studies ; Humans ; Critical Care* ; Interviews as Topic ; Korea ; Logistic Models ; Medical Records ; Mortality* ; Pupil ; Reflex ; Sodium ; Status Epilepticus