Twenty-two cases of idiopathic myocardiopathy were observed during the period of 1962 to 1971 and follow up clinical study was made in 5 cases. The criteria of diagnosis was based mainly on exclusive diagnosis in etiology unknown marked cardiomegaly. 1) Male and female ratio of idiopathic myocardiopathy was 1.1:1 and age of onset were distributed from first decade to fifth decade with similar number of cases. 2) Cardinal symptoms were dyspnea, palpitation, chest pain and cough. Common physical findings were protodiastolic gallop (in 2 cases), moist rales (in 3 cases) and hepatomegaly(in 3 cases). 3) Laboratory findings were normal except elevated T.T.T. in one case. 4) Electrocardiogram were abnormal in all cases. There were 3 cases of left ventricular hypertrophy, 3 cases of non-specific ST-T changes and one case of first degree A-V block, intraventricular conduction defect, abnormal Q wave and low valtage, respectively. 5) After medical treatment, symptoms were improved in 4 cases but heart size was reduced in only one case and E.C.G. abnormalities were unchanged in all cases.
Age of Onset ; Cardiomegaly ; Cardiomyopathies* ; Chest Pain ; Cough ; Diagnosis ; Dyspnea ; Electrocardiography ; Female ; Follow-Up Studies ; Heart ; Humans ; Hypertrophy, Left Ventricular ; Male ; Respiratory Sounds

BACKGROUND: Generalized immune activation occurs early in the course of many infectious disease. Laboratory investigations have shown that immune activation can be quantified by the measurement of soluble immune activation products in serum. Soluble CD25, CD8, and CD4 are major immune activation products. Soluble CD8 and CD4 are indices of CD8+ T cell and CD4+T cell activity, respectively. OBJECTIVE: We estimated the concentrations of these molecules in patients with leprosy. METHODS: The study population consisted of 31 patients with tuberculoid leprosy and 71 patients with lepromatous leprosy(32 cases of M. leprae negative patients and 39 cases of M. leprae positive patients). Serum samples and clinical and laboratory data were collected form each patient and control. The levels of serum soluble CD25, CD8, and CD4 were measured by sandwich enzyme immunoassay. RESULTS: The levels of serum soluble CD25 were significantly raised in leprosy patients as compared to control and did not vary signficantly between tuberculoid and lepromatous leprosy. The soluble CD8 levels in the serum of patients with leprosy did not differ from the levels of the control. The levels of serum soluble CD4 were significantly decreased in the patients with lepromatous leprosy, but not in the patients with tuberculoid leprosy. However, there was no significant correlation between CD25, CD8, and cD4 and bacterial indices in patients with lepromatous leprosy. CONCLUSIONs: There data suggest that non-specific immune activation occurs the spectrum in leprosy, while CD4+ T cell activity is significantly decreased in patients with lepromatous leprosy.
Communicable Diseases ; Humans ; Immunoenzyme Techniques ; Leprosy* ; Leprosy, Lepromatous ; Leprosy, Tuberculoid

Adrenal cortical carcinoma is a rare disease both in adults and in children. Most of these tumors are functional, especially in children, producing endocrine syndromes such as virilization, Cushing syndrome, hyperaldosteronism or feminization. We experienced a case of adrenal cortical carcinoma in a 7yr old boy who showed features of virilization such as rapid growth rate, penile enlargement, and pubic hair. This case was diagnosed with typical hormonal findings and abdominal MRI and confirmed by pathologic findings. He was successfully treated by total left adrenalectomy and has been followed up without problem over eight months.
Adrenalectomy ; Adrenocortical Carcinoma* ; Adult ; Child ; Cushing Syndrome ; Feminization ; Hair ; Humans ; Hyperaldosteronism ; Magnetic Resonance Imaging ; Male ; Rare Diseases ; Virilism

PURPOSE:Differential diagnosis of sexual precocity has been aided by new imaging techniques and endocrine evaluation. Sexual precocity, defined as the appearance of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys, was studied to evaluate the sex incidence and etiology of sexual precocity. METHODS:A total 95 children(87 girls and 8 boys) who were diagnosed with sexual precocity at St. Mary's Hospital, Catholic University Medical College from 1984 to 1996, were studied. All underwent standard anthropometric measures. They were assinged to diagnostic categories on the basis of clinical assessment, endocrine evaluation, radiologic imaging, and pelvic ultrasonography. RESULTS: 1) The most common cause of sexual precocity was premature thelarche, 50 cases(52.6%) of all children. 2) Central precocious puberty was noted in 7 girls and 3 boys(female to male ratio 2.3:1). The causes were not identified in 6 cases(85.7%) in girls, but the causes were not found in 1 cases(33.3%) in boys. The idiopathic central precocious puberty occurs at least two fold more frequently in girls than in boys. 3) Incomplete precocious puberty was noted in 29 girls and 5 boys(female to male ratio 5.8:1). In the causes of 29 girls with incomplete precocious puberty, congenital adrenal hyperplasia were found in 23 cases, autonomous functional ovarian cyst in 5 cases and McCune-Albright syndrome in 1 case. In the causes of 5 boys with incomplete precocious puberty, virilizing adrenal cortical tumor were found in 3 cases, congenital adrenal hyperplasia in 1 case, and hepatoblastoma in 1 case. CONCLUSION: Sexual precocity occurs at least ten fold more frequently in girls than in boys. Premature thelarche is the most common form of sexual precocity.
Adrenal Hyperplasia, Congenital ; Child ; Diagnosis ; Female ; Fibrous Dysplasia, Polyostotic ; Hepatoblastoma ; Humans ; Incidence* ; Male ; Ovarian Cysts ; Puberty, Precocious ; Ultrasonography

PURPOSE:Hypochondroplasia is a skeletal dysplasia characterized by poor childhood growth and an inadequate pubertal growth spurt. Final height attainment of hypochondroplasia has been reported to range between 120 and 152cm. Increased availability of growth hormone with the introduction of recombinant human growth hormone has allowed for clinical trials in a number of growth hormone sufficient children with growth problems. The purpose of this study was to assess the growth promoting effect of human growth hormone in children with hypochondroplasia. METHODS:Five patients with hypochondroplasia diagnosed by clinical and radiological findings between 1993 and 1997 at our hospital was aged 3 and 1/2 -11 and 1/2 years. Each patients continuously received human growth hormone 0.6-0.7U/Kg/week, intramuscularly or subcutaneously in 6-7 divided dose for 2 years. Standard auxologic assessment was carried out every 3 month interval in the first year after commencement of therapy and then same assessment was 6 monthly. Bone age was assessed 6 monthly using Gleurich-Pyle method. RESULTS:Mean height velocity of pretreatment and year 1 and 2 of GH treatment were 3.9+/-0.7, 6.5+/-1.8 and 5.7+/-1.5cm/year, respectively. Mean height standard deviation score for chronological age of pretreatment and year 1 and 2 of GH treatment were -2.7+/-0.3, -2.4+/-0.3 and -2.2+/-0.4, respectively. The increase in the height velocity diminishes over the subsequent year. The increment of bone age after GH treatment were same as the increments of chronological age. CONCLUSION: Short-term GH therapy increases the height velocity of children with hypochondroplasia, but the effect of GH therapy on final height remains unknown.
Child* ; Growth Hormone* ; Human Growth Hormone ; Humans

The hyperimmunoglobulinemia E (Jobs) syndrome (HIES) is characterized by marked elevated levels of IgE, recurrent cutaneous and systemic staphylococcal infections, atopic-like dermatitis, and defective neutrophil chemotaxis. Three cases of HIES have been reported in Korea, but not in the dermatology literature. We report a case of HIES with cutaneous infections and MRSA (methicillin-resistant Staphylococcus aureus). A 15-month-old girl presented with intractable pruritic excoriated papular pustular skin lesions and multiple subcutaneous abscesses. Surgical drainage of the abscesses and a course of antibiotic treatment in addition to topical steroids for about 7 weeks resulted in a remarkable improvement.
Abscess ; Chemotaxis ; Dermatitis ; Dermatology ; Drainage ; Female ; Humans ; Hypergammaglobulinemia* ; Immunoglobulin E ; Infant ; Job Syndrome* ; Korea ; Methicillin-Resistant Staphylococcus aureus ; Neutrophils ; Skin ; Staphylococcal Infections ; Staphylococcus ; Steroids

OBJECTIVE Present study was aimed to verify the effect of granulocyte macrophage-colony stimulating factor (GM-CSF) in the preimplantation development of mouse embryos and the involvement of the mitogen activated protein kiase (MAPK) in the GM-CSF signaling. METHODS: Two-cell embryos were cultured for 96 h in the presence or absence of GM-CSF (0, 0.4, 2, 10 ng/ml) and PD98059, a MEK inhibitor (10 muM). Morphological development, cell number per blastocyst, and apoptotic nuclei, were eamined. MAPK activity of embryonic immunoprecipitate by MAPK (ERK1/2) antibody was measured by in vitro phosphorylation of myelin basic protein. RESULTS: At post hCG 122 h the embryonic development among the experimental groups was significantly different (p=0.018). The rate of blastocyst development and cell number per embryo were the highest in 2 ng/ml GM-CSF treatment group. The percent of apoptotic cells of the GM-CSF-treated embryos was the lowest among the group. in blastocysts, GM-CSF treatment transiently increased MAPK activity. PD098059 attenuated the effect of GM-CSF on the morphological development, increase in cell number per blastocyst, down regulation of apoptosis, and upregulation of MAPK activity, suggesting that activation of MAPK cascade possibly mediated the embryotropic effect of GM-CSF. CONCLUSION: This result suggested that GM-CSF potentiated the development of preimplantation mouse embryos by activation of MAPK.
Animals ; Apoptosis ; Blastocyst ; Cell Count ; Down-Regulation ; Embryonic Development ; Embryonic Structures* ; Female ; Granulocyte-Macrophage Colony-Stimulating Factor ; Granulocytes* ; Mice* ; Myelin Basic Protein ; Phosphorylation ; Pregnancy ; Up-Regulation

Authors observed the antihypertensive effect of debrisoquin sulfate in 35 cases of essential hypertension and following results were obtained. 1) Results of short term control of blood pressure with debrisquin in 35 hypertensive patients revealed good control in 74.3%, fair in 14.3%, poor in 5.7% and failure in 5.7% of cases. Significant reduction of blood pressure was achieved in 7 of 9 cases of hypertension with addition of hydrochlorthiazide to debrisoquin. 2) During long term therapy with debrisoquin (duration of average 3.9 months), good control cases were slightly reduced(good in 58.6%, fair in 24.1%, poor in 6.9%, failure in 10.3%). But average effective dose of debriioquin was not increased significantly. 3) Antihypertensive effect of debrisoquin was slightly reduced in the cases of severe hypertension, hypertension of over 10 years duration and complication of cerebrovascular accident. 4) The side effects during treatment with debrisoquin were dizziness in 31.4%, headache in 11.4%, dry mouth, blurred vision, general weakness, insomnia in 8.6% respectively. There were no side effects in 48.6% of cases.
Blood Pressure ; Debrisoquin* ; Dizziness ; Headache ; Humans ; Hypertension* ; Mouth ; Sleep Initiation and Maintenance Disorders ; Stroke

PURPOSE: Thyroid disease is the most common endocrine disease in childhood. Thyroid hormone has critical effects on growth and development, especially in childhood. We survey the prevalence, sex and age distribution, symptoms and thyroid function states of thyroid diseases in childhood. METHODS: Three hundred ninety one children who were diagnosed as having thyroid disease at department of pediatrics, St. Mary's hospital from Jan. 1987 to Dec. 1998 enrolled in this study. RESULTS: 1) The ratio between male and female patients was 1: 5.3. Age distribution was puberty, school age and infancy in their order of frequency. 2) The results of thyroid function tests showed normal function in 210 cases (53.7%), increased function in 95 cases(24.3%), and decreased function in 86 cases (22%). 3) Simple goiter, Graves disease, and chronic lymphocytic thyroiditis were the most common diseases in euthyroid, hyperthyroid, and acquired hypothyroid state, respectively. 4) The most common sign and symptom was goiter in euthyroid(100%), hyperthyroid(98.8%) and acquired hypothyroid state(96.2%). Forty of 68 cases(58.8%) with congenital hypothyroidism were detected by neonatal screening. CONCLUSION: Simple goiter, chronic lymphocytic thyroiditis and Graves disease were common acquired thyroid diseases in childhood, and goiter is the most common clinical manifestation in acquired thyroid disease. Recently, increasing number of congenital hypothyroidism was detected by neonatal screening test.
Adolescent ; Age Distribution ; Child ; Congenital Hypothyroidism ; Endocrine System Diseases ; Female ; Goiter ; Graves Disease ; Growth and Development ; Hashimoto Disease ; Humans ; Infant, Newborn ; Male ; Neonatal Screening ; Pediatrics ; Prevalence ; Puberty ; Thyroid Diseases* ; Thyroid Function Tests ; Thyroid Gland*

PURPOSE: To demonstrate the characterization calretinin-immunoreactive displaced amacrine cells in the ganglion cell layer using immunohistochemistry and electron microscopy. METHODS: For immunohistochemistry, sections from guinea pig retina were incubated with mouse monoclonal antibody directed against calretinin. For double label studies, sections were incuated in mixture of mouse monoclonal anti-calretinin or rabbit polyclonal anti-calretinin with following antibodies: goat polyclonal anti-ChAT, rabbit polyclonal anti-GABA, mouse monoclonal anti-GABAA receptor alpha1, beta2/3. Sections were analyzed using Bio-rad Radiance Plus confocal scanning microscope. Stained sections from three guinea pig were observed with transmission electron microscope. RESULTS: Calretinin immunoreactivity was present in displaced amacrine cells and ganglion cells gaving rise to processes ramified in the inner part of the inner plexiform layer in stratum 4. The same stratum was also occupied by the dendrites of ON-cholinergic amacrine cells. Double-labeling demonstrated that dendrites and cell bodies of displaced amacrine cells colocalized with ON-cholinergic amacrine cells and dendrites of ganglion cells directly overlapped with dendrites of ON-cholinergic amacrine cells. The synaptic connectivity was identified by electron microscopy. Ganglion cell dendrites received synaptic input from ON-cholinergic amacrine cell. GABAA receptor beta2/3 subunit bands cofaciculates the dendrites of displaced amacrine cell and ganglion cell that are juxtapose to the alpha1 subunit of GABAA receptor. CONCLUSIONS: These results indicate that ON-cholinergic amacrine cells modulate calretinin-labeled ganglion cell via GABAA receptor beta2/3 in the guinea pig retina.
Amacrine Cells ; Animals ; Calcium-Binding Protein, Vitamin D-Dependent ; Dendrites ; Electrons ; Ganglion Cysts ; Goats ; Guinea ; Guinea Pigs ; Immunohistochemistry ; Mice ; Microscopy, Electron ; Retina