Congenital hypohidrotic ectodermal dysplasia is a rare genodermatosis. It is characterized by hypohidrosis hypotrichosis, dental hypoplasia and characterstic facial features, which reflect a wide constellation of developmental defec of tissue from the ectoderm. We have encountered three cases of congenital hypohidrotic ectodermal dysplasia in a 28-year-old female, her new-born baby, and a 10-month-old boy with a family history. All of the three patients had hypohidrosis, hypotrichosis, defective dentition, and characterstic facial features, which were characterstic features of this disorder. In addition, they showed dry skin, sparse and thin hairs. Histopathologic findings of previous cases revealed no eccrine gland structure in the dermis with routine and immunohistochemical stainning such cytokeratin and filaggrin. We report three typical cases of hypohidrotic ectodermal dysplasia with the review of literature.
Adult ; Dentition ; Dermis ; Eccrine Glands ; Ectoderm ; Ectodermal Dysplasia 1, Anhidrotic* ; Female ; Hair ; Humans ; Hypohidrosis ; Hypotrichosis ; Infant ; Keratins ; Male ; Skin

Van Wyk-Grumbach Syndrome is an advanced sexual development in association with primary hypothyroidism. The clinical feature in this syndrome is more consistent with stimulation of the FSH receptor by the markedly elevated TSH levels. Treatment of the hypothyroidism results in a rapid return to normal of the biochemical and clinical manifestations. We experienced a case of Van Wyk-Grumbach Syndrome and report with the brief review of related literature.
Hypothyroidism ; Puberty, Precocious ; Receptors, FSH ; Sexual Development

Pancoast syndrome (PS) is characterized by a malignant neoplasm of the superior sulcus of the lung with destructive lesions of the thoracic inlet and involvement of the brachial plexus and cervical sympathetic nerves. The most common initial symptom of PS is shoulder pain; however, cough, dyspnea, and hemoptysis, signs often associated with lung cancer, are not as common. Investigation of PS can be difficult even with plain radiographs of the chest because it is surrounded by osseous structures such as the ribs, vertebral bodies, and manubrium. Due to these characteristics, orthopedic surgeons tend to make a misdiagnosis resulting in delay of appropriate treatment. Here we report on a patient who was supposed to undergo rotator cuff repair for his shoulder pain and weakness, and was eventually diagnosed with PS.
Bays ; Brachial Plexus ; Cough ; Diagnostic Errors ; Dyspnea ; Hemoptysis ; Humans ; Lung ; Lung Neoplasms ; Manubrium ; Orthopedics ; Pancoast Syndrome* ; Ribs ; Rotator Cuff* ; Shoulder Pain ; Superior Vena Cava Syndrome ; Thorax

Profound reduction of anterograde coronary flow with concomitant ischemia is seen occasionally during percutaneous coronary intervention despite technically successful procedure. We found interesting coronary flow pattern in a patient with acute myocardial infarction, showing angiographic no reflow phenomenon after direct PTCA. The coronary blood flow pattern of the angiographic no-reflow phenomenon in this case was characterized by minimal systolic flow and sharp deceleration of diastolic flow. Coronary flow reserve calculated by the ratio of adenosine induced maximal hyperemic velocity and basal velocity was reduced. The Dopplertipped guide wire was useful for observation of phasic coronary flow pattern of angiographic no-reflow phenomenon.
Adenosine ; Deceleration ; Humans ; Ischemia ; Myocardial Infarction* ; No-Reflow Phenomenon ; Percutaneous Coronary Intervention

Management of unstable angina has evolved progressively. Although coronary angioplasty has recently shown to be relatively safe and effective treatment strategy for unstable angina, aute occlusion due to intracoronary thrombus accumulation during or immediately after coronary angioplasty remains to be one of the most common serious complication of this procedure. Intracoronary urokinase has been used to treat flow-limiting intracoronary thrombus accumulation that complicated initial successful percutaneous transluminal coronary angioplasty(PTCA) in unstable angina patient, which made the patient stablilzed. Thus in patient with flow-limiting intracoronary thrombus accumulation complicating PTCA, intracoronary urokinase proved to be highly effective in restoring vessel patency and preventing acute myocardial infarction. We report a case of successful revascularization with intracoronary infusion of urokinase in patient with intracoronary thrombus accumulation that complicated PTCA.
Angina, Unstable ; Angioplasty ; Angioplasty, Balloon, Coronary* ; Humans ; Myocardial Infarction ; Thrombosis ; Urokinase-Type Plasminogen Activator

In order to review the epidemiological data of cardiovascular diseases, statistical observation was made on the 7,271 patients with cardiovascular diseases who were treated in the Department of Internal Medicine, Han-Yang University Hospital during the 10 year period from May 1972 to December 1981. A. General Findings (1) Cardiovascular disease was seen in 18.6% out of a total 39,004 medical inpatients treated during this 10 year time span. (2) Incidences of cardiovascular disease seemed to increase in frequency in consecutive years, especially incidence of coronary heart disease and arrhythmia. (3) Incidence of cardiovascular disease occurred most frequently to patients in their fifth decade, and next most frequently to those in their sixth and fourth decade, in that order. (4) The overall ratio of male to female incidence of cardiovascular disease was 1:1. B. Specific Epidemiology (1) Hypertension was the most common form of cardiovascular problem, accounting for 52.9% of patients with cardiovascular disease and 9.9% of the total medical in-patients population. Incidence of hypertension occured most frequently in the fifth decade of life. It occured next most in the sixth and fourth decades, in that order. The male to female ratio of incidence of hypertension was 1:1. Patients with malignant hypertension made up 5.9% all hypertension patients. (2) Patients with rheumatic heart disease made up 6% of those patients with cardiovascular diseases. Male to female ratio of incidence of rheumatic heart disease was 1:2.4. The most common form of this disease was mitral valvular disease, but isolated aortic valvular disease proved to be rather uncommon in this study in Korea. (3) Coronary heart disease made up 15.5 % of all incidence of cardiovascular diseases. It occured most frequently to patients in their fifth and sixth decades, and in a male to female ratio of 1.5 to 1. (4) Congenital heart disease made of 0.9% of the total incidence of cardiovascular diseases with ventricular septal defect as the most common form of this disease. (5) The remaining forms of cardiovascular diseases diagnosed during the time span of this study were arrhythmia 16.8%, cor pulmonale 1.4%, cardiomyopathy 1.3%, peripheral vascular disease 1.0%, pericarditis 1.0%, thyrotoxic heart disease 0.8%, endocarditis 0.5%, postpartum cardiomyopathy 0.3%, myxedematous heart disease 0.1%, and miscellaneous heart ailments 1.5%.
Arrhythmias, Cardiac ; Cardiomyopathies ; Cardiovascular Diseases* ; Coronary Disease ; Endocarditis ; Epidemiology ; Female ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Heart Septal Defects, Ventricular ; Humans ; Hypertension ; Hypertension, Malignant ; Incidence ; Inpatients ; Internal Medicine ; Korea* ; Male ; Pericarditis ; Peripheral Vascular Diseases ; Postpartum Period ; Pulmonary Heart Disease ; Rheumatic Heart Disease

Hemangioma in the esophagus is an uncommon tumor. There have only been about 30 cases reported in the world literatures. It occurs predominantly in men and although majority are asymptomatic, may cause bleeding and dysphagia. Hemangioma in the esophagus was diagnosed with a barium swallowed esophagography and endoscopy. The main treatment modes recommended are surgery and endoscopic resection. We experienced one case of cav ernous hemangioma occurring at the distal esophagus. The patient was a forty-six year old male with dysphagia and indigestion. Barium esophagogram showed a filling defect at the distal portion. Esophagoscopy showed a bluish polypoid mass. Surgical resection was per formed and the pathologic diagnosis was confirmed as cavernous hemangioma. Postoperative course was uneventful and the patient had been followed up without any problems.
Barium ; Deglutition Disorders ; Diagnosis ; Dyspepsia ; Endoscopy ; Esophageal Neoplasms ; Esophagoscopy ; Esophagus* ; Hemangioma ; Hemangioma, Cavernous* ; Hemorrhage ; Humans ; Male

Clinical findings, electrocardiographic voltage and left ventricular function were evaluated in 104 patients with percardial effusion. Patients were classified into three groups by the amounts of pericardial effusion measured by two-dimensional echocardiography. 49 patients had small pericardial effusion, 31 moderate effusion and 24 large effusion. In 47 patients the effusions were clinically unsuspected prior to echocardiographic examination. Pericardial friction rub was noted in 14 patients, two-thirds of whom had moderate to large effusions. There was no relationship between the size of effusion and the presence of friction rub. Total QRS amplitudes were lower in patients with moderate to large effusions than those in control group. Maximum diastolic endocardial velocity(DEVM) and E-F slope of the anterior mitral leaflet were decreased significantly in patients with moderate to large effusions as compared with those with small effusions. As a result, authors concluded that reduction in the QRS voltages in serial ECGs may suggest the presence and the amount of pericardial effusion and also DEVM and E-F slope of the anterior mitral leaflet can be sensitive measures of left ventricular relaxation in patients with pericardial effusion.
Echocardiography* ; Electrocardiography* ; Friction ; Humans ; Pericardial Effusion* ; Relaxation ; Ventricular Function, Left*

Left ventricular hypertrophy(LVH) is one of common cardiovascular complications in hypertensive patients and it is well known that hypertensive cardiac disease accompained by LVH is still common cause of congestive heart failure in spite of treatment of hypertension. The authors assessed the prevalence of anatomical and functional abnormalities of left ventricle by EKG, chest X-ray and echocardiography in 45 essential hypertensive patients and also in 20 normal controls. Average values of left ventricular posterior wall thickness(LVPWd), interventricular septal thickness(IVSd), left ventricular mass(LVM), and left ventricular mass index(LVM/BSA) by echocardiography in hypertensive groups with LVH by EKG or chest X-ray were significantly higher than those of hypertensive groups without LVH by EKG or chest X-ray(P<0.005). Among 27 hypertensive patients with LVH by EKG and chest X-ray increased LVPWd was found in 24 patients(18%) and increased LVH in 26 patients(19%). Increased LVPWd and LVM were found in 3 patients(23%) among 13 hypertensives without LVH by EKG and chest X-ray. Hypertensive patients with increased LVH showed LVH by EKG and chest X-ray more frequently than those with increased LVPWd. Also, hypertensive patients without increased LVM showed MVH by EKG and chest X-ray less frequently than those without increased LVPWd. Therefore, echocardiography appears to be superior to routine chest X-ray and EKG for defecting LVH in hypertensive patients, especially without LVH by these tests. In conclusion, even though estimation of LVM by echocardiography seems to be a better method than single measurement of LVPWd, it seems thant estimation of LVM together with LVPWd will be more valuable in diagnosis of LVH in hypertensive patients.
Diagnosis ; Echocardiography* ; Electrocardiography ; Heart Diseases ; Heart Failure ; Heart Ventricles ; Humans ; Hypertension* ; Hypertrophy, Left Ventricular* ; Prevalence ; Thorax

BACKGROUN: B-type natriuretic peptide (BNP) is a cardiac neurohormone secreted from ventricles as a response to ventricular volume expansion and pressure overload. Myocardial performance index (MPI) reflects cardiac systolic and diastolic function and increase in the patients with congestive heart failure (CHF). The purpose of this study was to evaluate the correlation of clinical status of CHF, BNP level, and MPI. METHODS: We followed 30 patients (mean age: 64 +/- 13) admitted with symptomatic New York Heart Association (NYHA) class III to IV CHF. Medical treatment was done. Biochemical study including BNP level and evaluation of left ventricular (LV) function including MPI by echocardiography were performed at admission and 2 months later. RESULTS: Mean BNP level increased to 488.64 +/- 519.85 pg/mL at admission with symptomatic CHF and significantly decreased to 319.56 +/- 385.39 pg/mL (p-value: 0.014) after clinical improvement. Mean MPI tended to decrease from 0.62 +/- 0.37 to 0.45 +/- 0.26. Readmission rate in 4 months was high in the patients with small decrease in BNP level. At admission, the patients with symptomatic CHF showed increased MPI despite of normal LV systolic function and tendency to decrease in MPI after clinical improvement. CONCLUSION: Increased BNP level and MPI of patients admitted with symptomatic CHF decreased with clinical improvement. The results suggest that BNP level and MPI might be useful to guide treatment of patients with CHF and changes in BNP level during treatment are predictors of early readmission.
Echocardiography ; Heart Failure* ; Heart* ; Humans ; Natriuretic Peptide, Brain* ; Plasma*