Myotonia is characterized by failure of the voluntary muscles to relax immediately and persistence of contraction following voluntery movement or mechanical and electrical stimulation, Myotonic dystrophy (myotonia dystrophica, myotonia atrophica, Steinert's disease) is a hereditary progressive muscular abnormality with dominent transmittance and is characterized by myotonia, muscular wasting and weakness, and frequent association of frontal baldness, testicular atrophy, cataracts, and other nonmuscular abnormalities, We report a case of myotonic dystrophy in a 38 year-old man who had myotonia, muscular wasting and weakness, testicular atrophy, and frontal baldness, Electrodiagnostic studies indicated typical myotonic dystrophy. A review of literature is included.
Alopecia ; Atrophy ; Cataract ; Electric Stimulation ; Muscle, Skeletal ; Myotonia ; Myotonic Dystrophy

No abstract available.
Tumor Lysis Syndrome*

We analysed 26 cases of pyogenic osteomyelitis of the spine who were treaed at Younsei University Hospital during 25 years from January 1952 to August 1977 by operative or conaervative treatment. The diagnosis of pyogenic osteomyelitis of the spine is not easy and very confused. The cause and entry of the offending organism is mainly by venous drainage from the genitourinary and lower extremity circulation and the next is direct spreading from the adjacent focus. The typical clinical symptom is severe pain and tenderness over the lesion accompanied by febril history and the clinical course is relatively rapid than tuberculosis of the spine. In blood examination leukocytosis is uncommon but it showed relative increase of neutrophils in 62%, and erythrocyte sedimentation rate in 89%. Radiological findings are narrowing of joint space, erosion and/or destuctive change of vertebral body, vertebral marginal sclerotic change, and new bone formation and less frequently abscess shadow. The results obtained from this study were as follows, 1) The incidence of pyogenic osteomyelitis of the spine among total cases of the pyogenic osteomyelitis was 2.5% and the ratio of the pyogenic osteomyelitis of the spine to the spine tuberculosis was 2.8%. 2) The sexual incidence was in 11 and 15 each male and female. and the avrage, prevalent age was 34 years of age. 3) The pathogenic organism was isolated in 20 cases from the diseased focus during surgical procedure; stapyhloccus in 16 cases, pseudomonas in two and in a case of E.coli and proteus each. 4) The lumber vertebrae was mostly involved, i.e.lumbar in 11 cases, cervical in four, thoracic in nine, lumbosacral and sacrum in one each. 5) The operative treatment was performed in 23 cases and conservative in 3 cases with the satisfatory result in all cases. 6) We conclude that the focal curettage with anterior interbody fusion through anterior approach was better than the conservative treatment.
Abscess ; Blood Sedimentation ; Curettage ; Diagnosis ; Drainage ; Female ; Humans ; Incidence ; Joints ; Leukocytosis ; Lower Extremity ; Male ; Neutrophils ; Osteogenesis ; Osteomyelitis ; Proteus ; Pseudomonas ; Sacrum ; Spine ; Tuberculosis

The onprovement of civil registration reguires continuous study rather than periodic efforts. More and better statistics, however, are urgently required to formulate development programs and planning, Data obtainable from the civil registration are usually marred by errors of omission which are difficult to correct. This study aimed at finding out the problems occurring when a set of crude birth registration data in a rural area is used. Data Sources of this study are : 1) For birth registration : government birth registration records obtained from myun office and other government offices. 2) for the actual number of births : birth and child records from the Kang Wha Community Health Project. The study area is Sunwon Myun and Naega Myun in Kang Wha Gun, Gyunggido. The reference period for the accumulated data is one full year : Jan. 1st 1975 - Dec. 31st 1975. Major findings are as follows : If the number of registered birth is compared with the actual number of births which occurred in the target area, the former os far greater than the latter. The general assumption usually is , that the actual number of exceeds the registered number of birth in Korea. The observation from this specific study in this specific target area, shows the opposite trend. The number of births which actually occurred during the year of 1975 in the study area namely 256. The difference comes mainly from the fact that many cases of births from other areas were registered in the target area. In other words birth is not registered where it occurred but where the permanent residence address is. Among 550 births registered in the target area 66% did not occur in the target area. Only one third of all registered births were registered within the legal period for birth registration which is 2 weeks. 34% of the registered births actually occurred in 1974, but were registered in 1975. In 55% of the cases a difference was observed between the actual data of birth and the registered data of birth. From the 256 births which occurred in the target area, only 153 births (59%) were registered at the myun office and the remaining 130 births (41%) were not resistered there in the year of study. 6% of the 550 cases listed as registered have no separate registration sheets. Nevertheless, they definitely have been registered in the birth list at the myun office. 3% of the 550 cases are not recorded in this list but have a separate registration sheet at the myun offices. In conclusion, birth registration data have many errors and problems. Their usefulness as a source for vital and other statistics should be reconsidered. A series of sound methological studies will be necessary to establish their actual usefulness. A continuous and permanent compulsory system of birth recording is needed.
Birth Certificates ; Child ; Humans ; Korea* ; Parturition* ; Information Storage and Retrieval

BACKGROUND: Tuberculosis itself causes not only lung parenchymal destruction but also pulmonary vascular damage. Secondary emphysema also causes pulmonary vascular damage, which can develop as a late sequela of pulmonary tuberculosis. Therefore, pulmonary circulatory impairment tends to be more severe in post-tuberculosis emphysema than in primary emphysema. In post-tuberculosis emphysema, the right ventricular function may play an important role. However, little information regarding the right ventricular function is available. The purpose of this study was to evaluate and compare the right ventricular function between post-tuberculosis emphysema and primary emphysema. METHOD: Post-tuberculosis emphysema(PTE) or primary emphysema(PE) was diagnosed by history, HRCT finding and pulmonary function. Twenty patients with post-tuberculosis emphysema were matched with 20 patients with primary emphysema according to both FEV1 and FVC. Arterial blood gas analysis and echocardiography were done at rest and immediately after symptom-limited exercise. The right ventricular function was evaluated with the right ventricular ejection fraction using a modification of Simpson's method. RESULTS: There was no significant difference inthe demographics and pulmonary function between the two groups. In post-tuberculosis emphysema, the PaCO2 was higher (42.9±4.7 vs 38.8±3.1 mmHg at rest ; 47.9±7.0 vs 41.1±5.9 mmHg after exercise ; p<0.01) and the right ventricular ejection fraction was lower (57.6±6.5 vs 61.4±4.7 % at rest ; 51.1±10.8 vs 59.8±6.6 % after exercise ; p<0.01) both at rest and after exercise. The PaO2 after exercise was also lower (65.7±12.6 vs 80.2%±14.4 mmHg, p<0.01), while the PaO2 at rest tended to be lower(82.9±12.0 vs 87.8±7.5, p>0.05). In both groups, right ventricular ejection fraction correlated with the PaO2 after exercise(PTE r=0.536, PE r=0.557), and the PaCO2 at rest(PTE r=-0.576, PE r=-0.588) and after exercise(PTE r=-0.764, PE r=-0.619). CONCLUSION: Impairment of the right heart function and gas exchange were more serious in post-tuberculosis emphysema than in primary emphysema, and gas exchange may be influenced by the right ventricular function in post-tuberculosis emphysema.
Blood Gas Analysis ; Demography ; Echocardiography ; Emphysema* ; Heart ; Humans ; Lung ; Stroke Volume ; Tuberculosis ; Tuberculosis, Pulmonary ; Ventricular Function, Right*

No abstract available.
Neurofibromatoses* ; Pseudarthrosis* ; Ulna*

No abstract available.
Humans ; Infant* ; Infant, Low Birth Weight* ; Infant, Newborn ; Jaundice* ; Phototherapy*

Multiple neurilerv,omas occurred i.n a 14-year-old boy with neurofibromatosis and right eye blindness. His father succumbed to neurofibromatosis at the age of 42. Histologically-proved neurilemornas and neurofibromas were noted on the face, neck, trunk and extremities, but no cafe au-Iait spots ohserved. There were nevus anemicus, hairy nevus and pigmented moles. The associated features in this patient includc muscular atrophies, weakness of the left hand, parest,hesia of the medial side of left forcarm and Dupuytrens contracture of left fourth finger.
Adolescent ; Blindness ; Dupuytren Contracture ; Extremities ; Fathers ; Fingers ; Hand ; Humans ; Male ; Muscular Atrophy ; Neck ; Neurilemmoma* ; Neurofibroma ; Neurofibromatoses* ; Nevus ; Nevus, Pigmented

A case of pachyonychia congcnita occurring in a 8 year-old boy is described. A few weeks after delivery, his mother noticed thickening of all the finger and toe nails of her baby. Family history was denied. In addition to characteristic features of pachyonychia congenita of all the nails, follicular keratotic papules were noted on the knees, elbows, buttocks and legs, and also tender thick calluses, on the soles with palnioplantar hyperhidrosis. There were three cornu cutaneurn on the popliteal and left antecubital fossae. Other associated fea.tures include a painful tense bulla at the left fourth finger, irnpetiginous patches on the anterior thighs, hoarseness and leukokeratosis linguae, sparseness of the scalp hairs, and granulosis rubra nasi accompanied by facial hyperhidrosis.
Bony Callus ; Buttocks ; Child ; Elbow ; Fingers ; Hair ; Hoarseness ; Humans ; Hyperhidrosis ; Knee ; Leg ; Leukoplakia ; Male ; Mothers ; Nails, Malformed* ; Pachyonychia Congenita* ; Scalp ; Thigh ; Toes ; Transcutaneous Electric Nerve Stimulation

No abstract available.
Plasminogen Activators* ; Plasminogen* ; Pulmonary Embolism*