Ehlers-Danlos syndrome, a heritable disorder of connective tissue by autosomal dominant mode, is very rare disease in this country. Authors described a typical case in 39 years old woman who had a daughter of 6 years old also affectect by this disorder. The patient had the characteristic hyperelasticity of the skin and hyperextensibility of the jonts especially at metacarpophalangeal joint of the hands. She also revealed hyperterolism and aortic insufficiency on X-ray and E.C.G. examination.
Adult ; Child ; Connective Tissue ; Ehlers-Danlos Syndrome ; Family Characteristics* ; Female ; Hand ; Humans ; Metacarpophalangeal Joint ; Nuclear Family ; Rare Diseases ; Skin

Recurrent epistaxis is not one of rare symptoms in children, the well-known causes of which are anatomical abnormalities of nasal cavity and systemic bleeding tendency. but, in the majority of cases of recurrent epistaxis, it is usually very difficult to find out their underlying causes, so that the treatment is only symptomatic control of nasal bleeding whenever epistaxis occurs, but it usually is impossible to manage against their underlying causes as a specific therapy. The authors considered the breakdown or weakness of vascular integrity of nasal capillary vessel wall as an important factor of recurrent epistaxis, and vitamin C, which has an important role for the synthesis of collagen fiber, could influence to the vascular integrity of nasal capillary vessels. To elucidate the relation between recurrent epistaxis and the status of vitamin C in the tissue of the patients, the authors performed urinary vitamin C loading test measuring urinary excretion of vitamin C with high-performance liquid chromatography by Sirota et al in 19 cases of control group and 32 cases of patients with recurrent epistaxis, whose underlying diseases were not identified. And also the authors administered 1.0 gm/day of vitamin C orally for the treatment of recurrent epistaxis and follow-up was done over 1 year from the beginning of treatment to evaluate the treatment response. The results were as follows: 1) The hemoglobin level was significantly decreased in patient group compared with that of control group (12.4+/-0.8 vs 9.0+/-3.2 gm/dl). 2) The results of urine loading test of vitamin C, expressed as the percent excreted ratio, revealed significantly decreased in the patient group compared to that of the control group (9.1+/-6.1% vs 13.6+/-7.9%). 3) After treatment with 1.0 gm/day of vitamin C orally, follow-up was possible in 23 cases of total 32 patients. Of the 23 patients, "excellent" therapeutic response were in 16 cases (69.6%), "good" response in 5 cases (21.7%), and "no response" only in 2 cases (8.7%). The overall treatment response were in 21 cases (91.3%). In conclusion, it seems that majority of the patients with idiopathic recurrent epistaxis in the deficient state tissue vitamin C so that administration of vitamin C will be one of the effective therapy.
Ascorbic Acid* ; Capillaries ; Child* ; Chromatography, Liquid ; Collagen ; Epistaxis* ; Follow-Up Studies ; Hemorrhage ; Humans ; Nasal Cavity ; Vitamins*

A review has been made of mortality trends in Korea from 1958 to 1907 analyzing the data by sex, age and cause of death. The crude death rates and age specific death rates were estimate6 by the model of N. Keyfitz life table which had teen developed by the data of the 1960's national census. The cause specific death rates shown in this article are based on the following: all deaths occurring in the death-registration are expressed as a numberator, while the denominator was estimated from the regular national census data by interpolation method. It is estimated that only an average of about 40% of deaths which occurred during a year were registered during 1958 to 1967. The validity and the reliability of the diagnosis of causes of death seem to be extremely poor in this country. Therefore the cause specific death rates in this article are aimed to reveal trends of causes of registered death and not for the actual level of death rates. For 10 years very interesting mortality trends were observed : 1. The trend in the crude death rates was downward slowly. 2. The estimated death rate for the infant in 1960 was still high up to 100 per 1,000. 3. The rates for mortality attributed to such infectious diseases as pneumonia, bronchitis, gastroenteritis and measles decreased an average 40-60%. 4. The death rates for over-all tuberculosis decreased only 9.8%. 50% of the decrease was contributed by those in the less-than-15 year age group. 5. The death rates for chronic diseases, such as vascular diseases affecting the central nervous system, malignant neoplasm, major heart diseases and all accidents rose about 40-60%. 6. The rank order of the 10 leading causes of death showed large changes over the years, except for pneumonia and tuberculosis which occupyed 1st and End places respectively. Vascular diseases affecting the central nervous system moved from 5th to 3rd place and malignant neoplasm from 6th to 4th palace. The major heart diseases moved from 10th to 6th place and all accidents from 10th to 7th place. On the other hand, gastroenteritis moved from 3rd to 5th place and influenza from 4th to 8th place.
Adolescent ; Bronchitis ; Cause of Death ; Censuses ; Central Nervous System ; Chronic Disease ; Communicable Diseases ; Diagnosis ; Gastroenteritis ; Hand ; Heart Diseases ; Humans ; Infant ; Influenza, Human ; Korea* ; Life Tables ; Measles ; Mortality* ; Pneumonia ; Tuberculosis ; Vascular Diseases

This report is a part of the health survey, which was carried out in the summer of 1973 on all adults older than 15 years of age, inhabiting in the sampled area. Thirteen villages out of 31 natural villages in Shindong Myon, Chunseong Gun, Kangwon Province, a typical rural area of Korea were randomly sampled. A little over 70 percent of the population sampled, or 1198 persons were examined. The health survey included laboratory screening tests, history taking, physical examination and consultation to specialists for selected cases. Diseases of circulatory system were classified by WHO ICD A-List (150). Hypertensive heart disease was defined by the criteria that was used in U.S. Health Survey, and hypertension by WHO criteria for the purpose of comparison. Results obtained were as followings: 1. The overall prevalence of the diseases of circulatory system per 100 adults examined was 8.6 for male and 9.9 for female. 2. Age specific prevalence of the diseases showed gradually increasing pattern as age advanced for both sexes. For example, the prevalence for 15~19 years old age group was 2.4/100 adults which increased to 24.8 when age reached over 60 years. 3. The most prevalent disease category, 3.6/100 adults, was A-88: other diseases of circulatory system that was consisted mainly of varicose vein and hemorrhoids; the second one was hypetensive heart disease, 2.4. 4. Female had higher prevalence than male in A-84: other form of heart diseases (4 times, arrhythmia, beriberi heart disease, and heart disease secondary to anemia), in chronic rheumatic heart disease (4.5 times), and in hypertensive heart disease (1.4 times); in other hand, cerebrovascular diseases (4.5 times) and diseases of arterioles and capillaries (1.5 times) occurred more frequently among males than females. 5. The proportion of hypertensive heart disease among persons with definite hypertension was 22.0% for male, 25.8% for female, and it increased gradually as age increased. Hypertensive heart disease among persons with borderline hypertension was 2.2%. Only 27% of hypertensive heart disease patients had previous treatment for hypertension. 6. The prevalence of definite hypertension per 100 adults examined (double blood pressure check with interval of 30~40 minutes) was 9.5 for male, 9.4 for female, with increasing pattern by advancing ages; marked increase occurred after age of 45 years for male and 35 years for female. The prevalence of hypertension in this study was much lower than that of the study on employees of Korea Electricity Comparny when compared with the same age groups. 7. Electrocardiographic findings on 105 heart disease suspects revealed left ventricular hyperspecific change in 4 persons. This study was supported by China Medical Board of New York Inc.
Adult* ; Arrhythmias, Cardiac ; Arterioles ; Beriberi ; Blood Pressure ; Capillaries ; China ; Electricity ; Electrocardiography ; Female ; Gangwon-do ; Hand ; Health Surveys ; Heart Diseases ; Hemorrhoids ; Humans ; Hypertension ; Korea ; Male ; Mass Screening ; Physical Examination ; Prevalence* ; Rheumatic Heart Disease ; Specialization ; Varicose Veins

This report is a part of the health survey, which was carried out in the summer of 1973 on all adults older than 15 years of age, inhabiting in the sampled area. Thirteen villages out of 31 natural villages in Shindong Myon, Chunseong Gun, Kangwon Province, a typical rural area of Korea were randomly sampled. A little over 70 percent of the population sampled, or 1198 persons were examined. The health survey included laboratory screening tests, history taking, physical examination and consultation to specialists for selected cases. Diseases of circulatory system were classified by WHO ICD A-List (150). Hypertensive heart disease was defined by the criteria that was used in U.S. Health Survey, and hypertension by WHO criteria for the purpose of comparison. Results obtained were as followings: 1. The overall prevalence of the diseases of circulatory system per 100 adults examined was 8.6 for male and 9.9 for female. 2. Age specific prevalence of the diseases showed gradually increasing pattern as age advanced for both sexes. For example, the prevalence for 15~19 years old age group was 2.4/100 adults which increased to 24.8 when age reached over 60 years. 3. The most prevalent disease category, 3.6/100 adults, was A-88: other diseases of circulatory system that was consisted mainly of varicose vein and hemorrhoids; the second one was hypetensive heart disease, 2.4. 4. Female had higher prevalence than male in A-84: other form of heart diseases (4 times, arrhythmia, beriberi heart disease, and heart disease secondary to anemia), in chronic rheumatic heart disease (4.5 times), and in hypertensive heart disease (1.4 times); in other hand, cerebrovascular diseases (4.5 times) and diseases of arterioles and capillaries (1.5 times) occurred more frequently among males than females. 5. The proportion of hypertensive heart disease among persons with definite hypertension was 22.0% for male, 25.8% for female, and it increased gradually as age increased. Hypertensive heart disease among persons with borderline hypertension was 2.2%. Only 27% of hypertensive heart disease patients had previous treatment for hypertension. 6. The prevalence of definite hypertension per 100 adults examined (double blood pressure check with interval of 30~40 minutes) was 9.5 for male, 9.4 for female, with increasing pattern by advancing ages; marked increase occurred after age of 45 years for male and 35 years for female. The prevalence of hypertension in this study was much lower than that of the study on employees of Korea Electricity Comparny when compared with the same age groups. 7. Electrocardiographic findings on 105 heart disease suspects revealed left ventricular hyperspecific change in 4 persons. This study was supported by China Medical Board of New York Inc.
Adult* ; Arrhythmias, Cardiac ; Arterioles ; Beriberi ; Blood Pressure ; Capillaries ; China ; Electricity ; Electrocardiography ; Female ; Gangwon-do ; Hand ; Health Surveys ; Heart Diseases ; Hemorrhoids ; Humans ; Hypertension ; Korea ; Male ; Mass Screening ; Physical Examination ; Prevalence* ; Rheumatic Heart Disease ; Specialization ; Varicose Veins

Lupus nephropathy shows a variable clinical course including remission, relapse and progression to renal failure. To examine the clinical or morphological parameters which might be related to the progression of lupus nephropathy, we have analyzed renal biopsies of 19 patients (M : F=5 : 14), who underwent repeated renal biopsies by morphology and morphometry. The average age of the patients was 22.8+/-9.0 years. The mean interval between two biopsies was 34.5+/-13.3 months. The first renal biopsies of these patients were diagnosed with WHO class IV (74%), class II (11%), class I (5%), class III (5%), and class V (5%). According to the clinical data the patients were divided into 3 groups, static, relapsing and progressive. At the time of the first biopsies, the amount of proteinuria in both the static and relapsing groups was significantly higher than that in the progressive group (P<0.05). The volume density of the renal cortical interstitium of the first biopsies in the progressive group was significantly greater than that in the static and relapsing groups (0.14+/-0.07 micrometer3/micrometer3 vs. 0.05+/-0.02 micrometer3/micrometer3, P<0.05; 0.14+/-0.07 micrometer3/micrometer3 vs. 0.05+/-0.04 micrometer3/micrometer3, P<0.05). The activity index of the second biopsies in the relapsing group was significantly higher than that in the static group (2.7+/-0.6 vs 1.2+/-1.0, P<0.05). In the progressive group, the percentage of glomeruli with global sclerosis and the volume density of the renal cortical interstitium in the second biopsies was elevated over the first biopsies (P<0.05). Half of the patients in the static and relapsing groups underwent a morphologic transformation on the second biopsy. However, of the 7 patients in the progressive group, only one showed a transformation from WHO class IV to class III, suggesting that the transformation is not related to the progression of lupus nephropathy. These results suggest that interstitial expansion and heavy proteinuria at the time of the first renal biopsy may bode for poor prognosis in lupus nephropathy. Furthermore, they suggest that an increased histologic activity index could be related to the relapse of the disease.
Biopsy* ; Humans ; Prognosis ; Proteinuria ; Recurrence ; Renal Insufficiency ; Sclerosis

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

No abstract available.
Spinal Muscular Atrophies of Childhood*

No abstract available.
Esophageal Atresia*

We studied the incidence and morphological variation of the metopic suture in Korean. Ninety-six adult Korean dried skulls and 141 cases of antero-posterior skull roentgenograms from age of 1 month to 80years old. We divided metopic suture into complete and incomplete type. Incidence of complete type was 8.3% in dried skulls. In roentgenograms the incidence was decreased according to aging, especially around 6-8 years old, and over 20 years old age group the incidence was 4.0%. The incidence of incomplete type was 32.3%.
Adult ; Aging ; Humans ; Incidence ; Skull ; Sutures*