Congenital leukemia is a rare disease developing within the first 4 to 6 weeks of life, whose natural history is quite different from that of childhood and adult leukemia. Leukemia cutis occurs in 25% to 30% of infants with congenital leukemia and is more frequently associated with acute myeloid leukemia than acute lymphocytic leukemia. We report a case of congenital leukemia cutis in which a walnut-sized, slightly erythematous, dome-shaped tumor that had developed at left frontal area since birth was confirmed by skin biopsy. A review and analysis of Korean literature pertaining specially to leukemia cutis in congenital leukemia is presented along with the report of an additional case.
Adult ; Biopsy ; Humans ; Infant ; Leukemia* ; Leukemia, Myeloid, Acute ; Natural History ; Parturition ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Rare Diseases ; Skin

Cardiovascular changes has been reported eating and digestion. Experimental studies showed an early rise in cardiac output during eating which was attributable to an increased heart rate. This changes reverted to baseline valuse at the end of eating. To investigate postprandial cardiac functional change, we studied 20 healthy young men(average age 27 years) nonivasively with BP measurement, electrocardiography and M-mode echocardiohrapy before, 30 minutes and 3 hours after a lunch(600 Kcal). There was no significant changes in mean blood pressure after eating. On echocardiography, diastolic and systolic left ventricular internal dimensions were 4.9+/-0.4 and 3.1+/-0.4cm before meal and 4.9+/-0.4 and 2.9+/-0.3cm 30 minutes after eating. There was an average increase of 9% in cardiac output 30 minutes after meal. Fractional shortening was 36.4+/-6.4% before meal and 40.4+/-6.1(p<0.05) 30 minutes after eating. Ejection fraction changed from 73.5+/-7.9% to 78.2+/-6.3%(p<0.05) at postprandial 30 minutes. Myocardial contractility index assessed by the ratio of systolic BP to end-systolic volume was 4.3+/-1.6mgHg/ml before and 5.3+/-2.2mmHg/ml 30 minutes after eating. Peripheral resistance index as the ratio of mean blood pressure divided by cardiac index changed from 2,536+/-1,120dynesdSdcm-5/m2 to 2,048+/-472dynesdSdcm-5/m2 at postprandial 30 minutes. Electorcardiographic study revealed no changes in ST SE segment, T wave and heart rate after a meal. In conclusion, the increase of cardiac output after a moderate meal in healthy young men was associated with an increase in myocardial contractility and a decrease in peripheral vascular resistance.
Blood Pressure ; Cardiac Output ; Digestion ; Eating ; Echocardiography* ; Electrocardiography ; Heart Rate ; Humans ; Male ; Meals* ; Vascular Resistance

Purpose: To investigate the limited response to aflibercept after switching to aflibercept in neovascular age-related macular degeneration (AMD). Methods: This retrospective study included 70 eyes with neovascular AMD that were initially treated with ranibizumab and then switched to aflibercept. The incidence and timing of the limited response to aflibercept were identified and visual outcome was compared between eyes with and without limited response. In addition, factors predictive of limited response were analyzed. Results: A limited response to aflibercept was noted in approximately 1/5 of the patients who underwent switching to aflibercept in neovascular AMD. Switching to aflibercept was performed at a mean of 16.2 ± 12.7 months after diagnosis. During the mean 34.7 months of follow-up after switching, limited response was noted in 15 eyes (21.4%) at a mean of 22.0 ± 13.9 months after switching. The degree of reduction in visual acuity was mean logMAR 0.34 ± 0.41 in eyes with limited response and mean 0.06 ± 0.20 in eyes without (p = 0.002). In addition, the duration between the diagnosis and the switching was shorter (p = 0.012), and the number of ranibizumab injections before switching was lower (p = 0.016) in eyes with limited response than in eyes without. Conclusions Patients who showed limited response to aflibercept after switching to aflibercept showed a worse visual outcome. The probability of having a limited response is higher when the switching is performed earlier.

Purpose: To investigate the limited response to aflibercept after switching to aflibercept in neovascular age-related macular degeneration (AMD). Methods: This retrospective study included 70 eyes with neovascular AMD that were initially treated with ranibizumab and then switched to aflibercept. The incidence and timing of the limited response to aflibercept were identified and visual outcome was compared between eyes with and without limited response. In addition, factors predictive of limited response were analyzed. Results: A limited response to aflibercept was noted in approximately 1/5 of the patients who underwent switching to aflibercept in neovascular AMD. Switching to aflibercept was performed at a mean of 16.2 ± 12.7 months after diagnosis. During the mean 34.7 months of follow-up after switching, limited response was noted in 15 eyes (21.4%) at a mean of 22.0 ± 13.9 months after switching. The degree of reduction in visual acuity was mean logMAR 0.34 ± 0.41 in eyes with limited response and mean 0.06 ± 0.20 in eyes without (p = 0.002). In addition, the duration between the diagnosis and the switching was shorter (p = 0.012), and the number of ranibizumab injections before switching was lower (p = 0.016) in eyes with limited response than in eyes without. Conclusions Patients who showed limited response to aflibercept after switching to aflibercept showed a worse visual outcome. The probability of having a limited response is higher when the switching is performed earlier.

From May, 1975 to August, 1986, we experienced 21 patients with ruptured aneurysm of the sinus of Valsalva. 1) Their ages ranged from 15 to 52 years with a mean age of 26.7 years, and 14 patients were male and 7 patients were female. Among 18 cases of which aneurysms occurred on the right sinus of Valsalva, 15 cases (83%) ruptured into the right ventricle, 1 case (6%) ruptured into the right atrium and 2 cases (11%) ruptured into the pulmonary artery. All 3 cases of which aneurysms occurred on the noncoronary sinus ruptured into the right atrium. 2) Clinical symptoms consisted of dyspnea (95%), palpitation (81%), chest pain (43%), orthopnea (33%), cough (14%), hemoptysis (5%), oliguria (5%), and fever (5%). In all patients continuous heart murmur was audible. Hepatomegaly (48%), pulmonary basal rale (14%), and pretibial pitting edema (19%) were also observed. 3) In all patients diagnosis was made with cardiac catheterization and angiography, and confirmed by operation excetpt one case. 4) Among 20 patients who had been performed corrective surgery, ventricular septal defect was observed in 16 patients (85%), aortic insufficiency in 5 patients (25%), pulmonary infundibular stenosis in 1 patient (5%), patent foramen ovale in 1 patient (5%), and tricuspid insufficiency in 1 patient (5%). Ventricular septal defect was observed in all 16 patients whose aneurysms of the right coronary sinus of Valsalva ruptured into the right ventricle and the pulmonary artery. 5) All patients survived operation, and no postoperative complication was found except 1 patient to whom reoperation was performed because of postoperative aortic insufficiency and detachment of the patch closure for ventricular septal defect. There was symptomatic improvement in all patients.
Aneurysm ; Aneurysm, Ruptured* ; Angiography ; Cardiac Catheterization ; Cardiac Catheters ; Chest Pain ; Coronary Sinus ; Cough ; Diagnosis ; Dyspnea ; Edema ; Female ; Fever ; Foramen Ovale, Patent ; Heart Atria ; Heart Murmurs ; Heart Septal Defects, Ventricular ; Heart Ventricles ; Hemoptysis ; Hepatomegaly ; Humans ; Male ; Oliguria ; Postoperative Complications ; Pulmonary Artery ; Pulmonary Subvalvular Stenosis ; Reoperation ; Respiratory Sounds ; Sinus of Valsalva*

PURPOSE: To evaluate the efficacy of an optical coherence tomography (OCT)-based diagnosis of polypoidal choroidal vasculopathy (PCV) in Korean patients. METHODS: This retrospective, observational case series included 263 eyes of 263 patients (147 eyes with PCV and 116 eyes with typical exudative, age-related macular degeneration [AMD]) who had been diagnosed with treatment naïve exudative AMD. Eyes with three or more of the following OCT findings were diagnosed with PCV: multiple retinal pigment epithelial detachment (RPED), a sharp RPED peak, an RPED notch, a hyporeflective lumen representing polyps, and hyperreflective intraretinal hard exudates. The OCT-based diagnosis was compared with the gold-standard indocyanine green angiography-based method. The sensitivity and specificity of the OCT-based diagnosis was also estimated. An additional analysis was performed using a choroidal thickness criterion. Eyes with a subfoveal choroidal thickness greater than 300 µm were also diagnosed with PCV despite having only two OCT features. RESULTS: In eyes with PCV, three or more OCT features were observed in 126 of 147 eyes (85.7%), and the incidence of typical exudative AMD was 16 of 116 eyes (13.8%). The sensitivity and specificity of an OCT-based diagnosis were 85.7% and 86.2%, respectively. After applying the choroidal thickness criterion, the sensitivity increased from 85.7% to 89.8%, and the specificity decreased from 86.2% to 84.5%. CONCLUSIONS: The OCT-based diagnosis of PCV showed a high sensitivity and specificity in Korean patients. The addition of a choroidal thickness criterion improved the sensitivity of the method with a minimal decrease in its specificity.
Aged ; Choroid/blood supply/*diagnostic imaging ; Choroid Diseases/*diagnosis/epidemiology/physiopathology ; Diagnosis, Differential ; Female ; Fluorescein Angiography ; Follow-Up Studies ; Fundus Oculi ; Humans ; Incidence ; Male ; Republic of Korea/epidemiology ; Retrospective Studies ; Tomography, Optical Coherence/*methods ; Visual Acuity

Among the series of 272 cases of surgically treated anterior circulation aneurysms, we experienced 6 cases of central infarctions involving caudate nucleus, globus pallidus, putamen and genu of internal capsule respectively or in combination. These surgery related complications were caused by the injury to the perforators going to the anterior perforated substance during manipulation of the large aneurysm or the aneurysms ruptured prematurely. The clinical courses of these patients, however, were not so severe. The morbidities were minimal or none after the average follow-up periods of 17 months. Thorough knowledge of the anatomy of the perforators may help to minimize the severe morbidity in the management of large or difficult aneurysms.
Aneurysm* ; Caudate Nucleus ; Follow-Up Studies ; Globus Pallidus ; Humans ; Infarction* ; Internal Capsule ; Olfactory Pathways ; Putamen

We experienced 3 cases of Candida esophagitis in infancy which were diagnosed by esophageal endoscopy. First case, 10 month-old boy with combined immune deficiency had suffered from oral thrush and poor feeding for more than 4 months. Esophageal endoscopy revealed multiple whitish creamy patches on the friable erythematous and necrotic mucosa of the esophagus. He was firstly treated with amphotericin-B but in vain. Then he was treated with fluconazole (5 mg/kg/day) and in a few days oral thrush nearly disappeared and endoscopy after 2 weeks revealed complete healing of the esophagitis. Second case, 6 month-old boy with some cellular immue defect also suffered from oral thrush, poor feeding and intermittent fever. He was treated with fluconazole and oral thrush was imporved. He was discharged without follow up endoscopy. Third case, 4 month-old girl with liver cirrhosis due to infantile cholestasis had Candida sepsis. Esophagitis was found incidentally during the endoscopic examination of esophageal varix. First 2 cases showed multiple small filling defects and decreased motility on esophagography. Candida antigen was not detected in the sera of all 3 cases of candidiasis. We conclude that Candidia esophagitis should be suspected when an infant has been suffering from long-term treatmet-resistant oral thrush and poor feeding and that esophageal endoscopy can be easily performed in infants also and useful in diagnosing esophagitis and assessing the outcome of treatment.
Candida* ; Candidiasis ; Candidiasis, Oral ; Cholestasis ; Endoscopy ; Esophageal and Gastric Varices ; Esophagitis* ; Esophagus ; Female ; Fever ; Fluconazole ; Humans ; Infant ; Liver Cirrhosis ; Male ; Mucous Membrane ; Sepsis

Solitary fibrous tumor is a spindle cell neoplasm that can arise in any place of the body. Intracranial solitary fibrous tumors are rare. To our knowledge, only 57 cases with intracranial lesion have been reported. In Korea three cases have been reported. Our case was a 23-year-old woman who presented with morning headache. MRI showed a large intra-axial mass involving falx with typically isointense and heterogeneous strong enhancement on T1 weighted image in the right parieto-occipital region. Histologically the tumor showed spindle shaped cells within matrix with thick collagen deposition, hypercellularity, focal necrosis, and pleomorphism. Immunohistochemical study demonstrated diffuse positivity for CD34, Vimentin, Reticulin. In case of the intracranial tumors involving the meninges, we also should consider the solitary fibrous tumor with immunohistochemical staining for accurate diagnosis.
Collagen ; Diagnosis ; Female ; Headache ; Humans ; Korea ; Magnetic Resonance Imaging ; Meninges ; Necrosis ; Reticulin ; Solitary Fibrous Tumors* ; Vimentin ; Young Adult

Primary Sjogren's syndrome is a chronic autoimmune disorder characterized by xerostomia and keratoconjunctivitis sicca result from lymphocytic infiltrations of salivary and lacrimal glands.It may accompany by the wide spectrums of extraglandular symptoms,such as musculoskeletal,pulmonary,renal,intestinal,hematologic,and nervous system.However,overt cardiac manifestations are very rare and are documented only a few cases sporadically. We report a patient with primary Sjogren's syndrome who developed reversible congestive heart failure as part of a myocarditis.A 39-year-old woman with primary Sjogren's syndrome developed fatigue,dyspnea,and abdominal discomfort.An echocardiography revealed global hypokinetic left ventricle with markedly decreased ejection fraction (EF=39%),abnormal restrictive filling pattern,secondary tricuspid valve insufficiency and no pericardial effusion or thickening.A myocardial biopsy disclosed myocardial degeneration with lymphocyte infiltration.Following treatment with digitalis,diuretics,ACE inhibitor and corticosteroid,the symptoms of congestive heart failure improved.A repeat echocardiography showed an improved ventricular contractility and recovered both diastolic (normalized E/A ratio)and systolic (EF=50%)functions.
Adult ; Biopsy ; Echocardiography ; Female ; Heart Failure ; Heart Ventricles ; Humans ; Keratoconjunctivitis Sicca ; Lymphocytes ; Myocarditis* ; Pericardial Effusion ; Sjogren's Syndrome* ; Tricuspid Valve Insufficiency ; Xerostomia