No abstract available.
Child* ; Human Growth Hormone ; Humans ; Kidney Failure, Chronic*

In the published version of this article, the contents of Table 1 (‘Demographic characteristics of subjects’) are incorrect.

Selective infusion of the epinephrine into the renal artery has been used in the field fo the diagnostic andthe therapeutic radiology for correct diagnosis and effective treatment, respectively. However, administration ofoverdose of epinephrine may cause serious complication, renal infarction. The study was undertaken to evaluate thesequential change of renal arterial constrictive effect of selective infusion of epinephrine into renal artery andto determine the critical doses of epinephrine producing irreversible renal infarct. A total of 25 rabbits areused, which are devided into 5 groups. Under the general anesthesia is made the selective infusion of variousdoses of epinephrine into the right renal arter of the rabbits. At the various time interval during and after theepinephrine infusion, renal angiography was doen, and 24 hrs. later, gross and microscopic findings of the kidneywere observed. The results are as follows; 1. Vasoconstriction of renal artery occurred within 2 mins. infusion,and maximum effect within 5 mins. 2. It seems that there is correlation between the amount of infused epinephrineand the time taken to recover from constiction of renal artery. 3. When epinephrine is infused into the renalartery in the rate of 1ug/min., renal infarct is not noticed below the level of 10 mins., but correlation betweenthe amount of infused epinephrine and the frequency of renal infarct occures above 20 mins. infusion.
Anesthesia, General ; Angiography ; Diagnosis ; Epinephrine ; Infarction ; Rabbits ; Radiation Oncology ; Renal Artery ; Vasoconstriction

During the period of 4 years from May, 1981 to April, 1985, 71 cases of obstructing subinfundibular muscular bundles of the right ventricle in congential cardiovascular anomalies were diagnosed by cariac catheterization and angiography at Hanyang University. The finding of right ventricular angiogram and degree of the right ventricular outflow obstruction, clinical data, electrocardiographic data and hemodynamic data were correlated irrespectively. The summary of this article is as follows. 1) The age of patients was ranged from 1 to 22 years old with a mean age of 8 years old. There were 39 males and 32 females with M:F ratio of 1.2:1. The incidence of obstructing subinfundibular muscular bundles of the right ventricle in congenital cardiovascular anomalies was higher with increasing age. 2) The associated cardic anomalies were as follows: 40 cases (56%) of isolated ventricular septal defect(VSD), 13(18%) of tetralogy of Fallot physiology, 7(10%) of patent ductus arteriosus(PDA), 3(4%) of pulmonary stenosis, 1 aortic stenosis, 1 double outlet of right ventricle(DORV), 1 trilogy, 1 ostium secundum defect, etc. The incidence of VSD with or without other associated cardiovascular anomalies was 56 cases(79%) out of 71 cases. 3) Maximum systolic pressure gradient between proximal and distal chamber of the right ventricle were under 25 mmHg in 32 cases, between 25 and 50 mmHg in 13 and above 50 mmHg in 26. Pressure gradients of all 7 cases with PDA were under 25 mmHg. 4) Correlative assesment of angiographic manifestation(2 indicies:Diameter of right ventricular outflow tract(systolic phase)/diameter of tricuspid valvular annulus(diastolic phase)=OT/TV, Diameter of right ventricular outflow tract(systolic phase)/Length of right ventricular diaphragmatic surface(systolic phase)=OT/RV) according to pressure gradient, OT/Tv and OT/RV values were lower the increasing pressure gradient, between proximal and distal chamber of the right ventricle. These were reverse correlations but coefficients of correlation(r) were-0.49and -0.48. Therefore, the degree of right ventricular outflow obstruction could be predicted, using 2 indices of right ventricular angiogram in individual cases, but could not be calculated accurately. 5) This indicated that pressuer gradient was also affected by technical errors, variable cardic anomalies, development of sinusoid, age and the other factors. 6) We assumed that non-invasive Doppler echocardiography could be useful in making the diagnosis and follow up of the patient with obstructing subinfundibular muscle bundles in right ventricle.
Angiography ; Aortic Valve Stenosis ; Blood Pressure ; Catheterization ; Catheters ; Child ; Diagnosis ; Echocardiography, Doppler ; Electrocardiography ; Equidae ; Female ; Heart Ventricles* ; Hemodynamics ; Humans ; Incidence ; Male ; Physiology ; Pulmonary Valve Stenosis ; Tetralogy of Fallot ; Ventricular Outflow Obstruction ; Young Adult

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

No abstract available.
Anemia, Iron-Deficiency* ; Iron* ; Milk*

We present the first case report of fragile X-associated tremor ataxia syndrome (FXTAS) in the Republic of Korea. A 75-year-old male developed progressive gait ataxia, parkinsonism, and a mood disorder. Magnetic resonance imaging revealed T2 high signal intensity within the middle cerebellar peduncles. Analysis of the fragile X mental retardation 1 gene revealed a CGG trinucleotide repeat number of 136. FXTAS should be considered when a patient has atypical parkinsonism, cerebellar ataxia, and specific MRI abnormalities.

Superficial siderosis is a rare condition characterized by hemosiderin deposition in leptomeninges, subpial tissue, brainstem, cerebellum, spinal cord, and cranial nerves. Slowly progressive hearing loss and gait ataxia are invariable clinical manifestations. We report three patients with their clinical and radiological features. All patients presented with hearing loss and cognitive dysfunction. Two showed gait ataxia and myelopathic symptoms and signs. Decreased visual acuity, hand tremor, limb ataxia, dysarthria, and nystagmus were also present. All patients showed typical MRI findings: marked linear hypointensities around the cerebellum, brainstem, and the surface of the cerebral cortex, especially in sylvian fissures. Two patients had brain tumors : pituitary adenoma and oligodendroglioma. Another patient had no definite bleeding source. Hemosiderin deposition is caused by chronic and recurrent subarachnoid hemorrhage derived from tumor, vascular malformation, aneurysm, posthemispherectomy, and unknown bleeding sources. Diagnosis is easily made by characteristic clinical manifestations and MRI findings. The selective vulnerability of the cerebellum and the 8th cranial nerve depends upon their own histological and biochemical characteristics. Benefits of the iron chelating agents are questionable. Removal of the possible bleeding sources is the most reliable strategy to prevent the disease progression.
Adenoma ; Aneurysm ; Ataxia ; Brain Neoplasms ; Brain Stem ; Cerebellum ; Cerebral Cortex ; Cranial Nerves ; Diagnosis ; Disease Progression ; Dysarthria ; Gait Ataxia ; Hand ; Hearing Loss ; Hemorrhage ; Hemosiderin ; Humans ; Iron Chelating Agents ; Magnetic Resonance Imaging ; Oligodendroglioma ; Pituitary Neoplasms ; Siderosis* ; Spinal Cord ; Subarachnoid Hemorrhage ; Tremor ; Vascular Malformations ; Visual Acuity

The importance of precise information and knowledge on the initial estimates (IEs) in modeling has not been paid its due attention so far. By focusing on the IE, this tutorial may serve as a practical guide for beginners in pharmacometrics. A 'good' set of IEs rather than arbitrary values is required because the IEs where NONMEM kicks off its estimation may influence the subsequent objective function minimization. To provide NONMEM with acceptable IEs, modelers should understand the exact meaning of THETA, OMEGA and SIGMA based on physiology. In practice, problems related to the value of the IE are more likely to occur for THETAs rather than the random-effect terms. Because it is almost impossible for a modeler to give a precise IE for OMEGAs at the beginning, it may be a good practice to start at relatively small IEs for them. NONMEM may fail to converge when too small IEs are provided for residual error parameters; thus, it is recommended to give sufficiently large values for them. The understandings on the roles, meanings and implications of IEs even help modelers in troubleshooting situations which frequently occur over the whole modeling process.
Physiology

A case of transient neonatal diabetes mellitus combined with congenital adrenal hyperplasia(CAH) is described. A female infant was born by cesarean delivery due to fetal distress, she had sunken eyeball and anterior fontanelle, large protruded tongue and thin subcutaneous tissues. She had large clitoris and progressive pigmentation on whole body was observed since 10th day of birth. Hyperglycemia and glycosuria was noted at 3rd day of birth. Level of insulin and C-peptide was 3.0 mU/L and 0.35 ng/mL respectively. Serum ACTH was 870.4 pg/mL and 17-hydroxyprogesterone was increased to 20,000 ng/dL. Serum Na was 124 mEq/L, K 5.6 mEq/L. Abdominal MRI showed no abnormality. Chromosomal study showed 46,XX. Genetic analysis with polymorphic DNA markers for chromosome 6 showed paternal uniparental isodisomy at D6S276, D6S1704 and DNA analysis of CYP 21 gene showed mutation at P435S. She required insulin therapy for 8 months after birth. Hydrocortisone and florinef was needed for the control of CAH.
17-alpha-Hydroxyprogesterone ; Adrenal Hyperplasia, Congenital* ; Adrenocorticotropic Hormone ; C-Peptide ; Chromosomes, Human, Pair 6 ; Clitoris ; Cranial Fontanelles ; Diabetes Mellitus* ; DNA ; Female ; Fetal Distress ; Genetic Markers ; Glycosuria ; Humans ; Hydrocortisone ; Hyperglycemia ; Infant ; Insulin ; Magnetic Resonance Imaging ; Parturition ; Pigmentation ; Subcutaneous Tissue ; Tongue ; Uniparental Disomy