The Arnold-Chiari malformation (ACM) is an anomaly of the hindbrain consisting of two components: a variable displacement of a tongue of tissue derived from the inferior cerebellar vermis in the upper cervical canal and a similar caudal dislocation of the medulla and fourth ventricle. Hydrocephalus and meningomyelocele are another abnormalities that associated frequently. We present a case of Arnold-Chiari malformation with hydrocephalus and meningomyeolcele and a brief review of the literatures was added.
Arnold-Chiari Malformation* ; Cerebellar Vermis ; Dislocations ; Fourth Ventricle ; Hydrocephalus ; Meningomyelocele ; Rhombencephalon ; Tongue

No abstract available.
Acanthosis Nigricans* ; Growth Hormone*

No abstract available.
Hyperhidrosis

The triad of bilateral optic atrophy, hearing deficit, peripheral neuropathy is knoun to be a rare disorder. The authors experienced eight patients in three generations of the same family with this triad of deficits. The disorder developed since their childhood and the course was slowly progressive. Nerve conduction study suggested peripheral neuropathy and sural nerve biopsy was compatible with demyelinating neuropathy, as there were reduction of myelinated nerve fibers in number and focal onion-bulb formation. The mode of inheritance of the family seems to be autosomal dominant, with relatively high penetrance. In many respects, the disorder resembles the cases reported by Sylvester(1958) and Iwashita et al.(1970). But still we do not know the exact etiology of this disorder.
Biopsy ; Deafness* ; Family Characteristics ; Hearing ; Humans ; Nerve Fibers, Myelinated ; Neural Conduction ; Optic Atrophies, Hereditary* ; Optic Atrophy ; Penetrance ; Peripheral Nervous System Diseases* ; Sural Nerve ; Wills

Malignant Mixed Mullerian tumors(MMMTs) are unusual neoplasms occumng mostly in the uterus and, ralely, they arise in the ovary. The clinical features of malignant mixed mullerian tumor of the ovary are similiar to other ovarian malignancies. The clinical course is rapidly progressive and fatal. The optimal treatment modalities has remained elusive. The most reliable prognostic criterion is the initial tumor stage and the overall survival was poor. We experienced two cases of malignant mixed miillerian tumor of the ovary, so we report these cases with a brief review of the concerned literatures.
Female ; Ovary* ; Respiratory Sounds ; Uterus

BACKGROUND: Quinidine appeared to increase serum digoxin levels when given with quinidine. Therefore elevated serum digoxin concentrations and clinical toxicity have been reported in patient receiving quinidine. Currently, Bayesian method which estimates the most probable parameters of the drug for each patient from population parameters data is useful approach for adjusting digoxin dosage. To increase the accuracy of Bayesian method, it is desirable to use population parameters of Korean. Therefore we evaluated the effect of quinidine on digoxin clearance in Korea. METHOD: Patient's records from 19 adult cardiac disease without CHF having normal renal and liver function from Seoul National University of Hospital respectively wre evaluated. Digoxin pharmacokinetic parameters, CL and Vd, were obtained from serum concentration of digoxin of single and combined therapy at each steady-state by using bayesian method. RESULTS: This study show that quinidine reduced the total body clearance of digoxin from 2.39+/-0.17 to 1.51+/-0.08ml/min/kg(p<0.05) and reduced the digoxin volume of distribution from 8.57+/-0.29 to 4.98+/-0.19L/kg(p<0.05). This results show that digoxin dosage reduced to 40-50% in Korean, if quinidine therapy is initiated.
Adult ; Bayes Theorem ; Digoxin* ; Drug Interactions ; Heart Diseases ; Humans ; Korea ; Liver ; Pharmacokinetics ; Quinidine* ; Seoul

Apgar score is most widely used evaluating indicator of newborn health, but it is very subjective. Umbilical cord blood gas analysis is more objective and effective than Apgar score in evaluation of newborn status. Cord blood gas was changed slightly by processing of time after fetal birth. This study was undertaken to observe objectiveness and effectiveness of umbilical arterial blood gas analysis and effects of time interval of cord clamping to newborn health with 122 pregnant women and their babies. We observed following results: 1. There were poor correlation between Apgar score and umbilical cord arterial blood analysis in evaluating of newborn health (P>0.05). 2. There was no clinical significance of Apgar score or umbilical arterial blood gas analysis as single indicator in evaluating of newborn health (P>0.05). 3. Gas analysis and pH of umbilical arterial blood was more helpful in evaluating of newborn health than Apgar score. 4. There were no significant effects of time interval of umbilical cord clamping to newborn health. 5. If there were no indications of early umbilical cord clamping, cord blood sampling at immediately after birth without cord clamping was more effective to evaluate newborn status.
Apgar Score* ; Blood Gas Analysis ; Constriction ; Cordocentesis ; Female ; Fetal Blood ; Humans ; Hydrogen-Ion Concentration* ; Infant Health* ; Infant, Newborn* ; Parturition ; Pregnant Women ; Umbilical Cord

No abstract available.
Neurofibromatoses* ; Peripheral Nerves*

From Nov. 7. 1988 to Nov. 21. 1988, we visited 20 universities' opticians and contact lens stores affiliated with them, and surveyed contact lens wearing process. We found that in all 14 stores, fitting was done by non-ophthalmologists and in 11 stores, wearing was processed without consulting on ophthalmologists.

Though the incidence of paragonimiasis has been remarkably decreased since 1970, it is still not a rare disease in Korea. Major problems in the diagnosis of pulmonary paragonimiasis on chest radiography are its differentiation from pulmonary tuberculosis and lung cancer. Chest radiographic findings have been described in detail, but little have been reported on CT findings. We reviewed CT findings of 10 patients with pulmonary paragonimiasis. The characteristic CT findings were similar to those on chest radiography, such as air-space consolidation (70%), nodular mass (50%), pleural effusion (40%), cystic lesion (30%), small low density within the mass (30%), linear density (20%), pneumothorax(20%), and burrow track (20%). CT depicted the cystic lesions and the burrow tracks more clearly and showed the small worm-retaining cysts within the mass that were not detectable on chest radiography. In conclusion, all of these CT findings are useful in the diagnosis of pulmonary paragonimiasis especially when differentiation from tuberculosis or lung canceris difficult on chest radiography.
Diagnosis ; Humans ; Incidence ; Korea ; Lung ; Lung Neoplasms ; Paragonimiasis* ; Pleural Effusion ; Radiography ; Radiography, Thoracic ; Rare Diseases ; Thorax ; Tuberculosis ; Tuberculosis, Pulmonary