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*

Pseudotumor cerebri is a condition of obscure etiology characterized by headache, papilledema and raised intracranial pressure in the absence of a space-occupying lesion. We have experienced a case of pseudotumor cerebral complicated by acute frontal sinusitis associated with mild intracranial meningeal inflammation. We have checked the intracranial pressure through serial lumbar puncture before and after medication and conservative management. Symptom were resolved gradually upon lowering the intracranial pressure by serial lumbar puncture and conservative management. So we report a case with brief review of the literature.
Frontal Sinus* ; Frontal Sinusitis* ; Headache ; Inflammation ; Intracranial Pressure ; Papilledema ; Pseudotumor Cerebri* ; Spinal Puncture

Undifferentiated(embryonal) sarcoma of the liver developing predominantly in the late childhood is extremely rare in malignant hepatic tumors. It has been described by a variety of terms such as malignant mesenchymoma, embryonal sarcoma, fibromyxosarcoma, embryonal rhabdomyosarcoma or primary sarcoma of the liver. The tumor usually presents a huge mass containing solid and sometimes cystic components, and is cmposed of large stellate and spindle cells in an abundant myxoid matrix. The treatment of choice is surgical resection with or without adjuvant chemotherapy and/or radiotherapy, but the prognosis of this disease has been reported extremly poor. We experienced a case of undifferentiated(embryonal) sarcoma of the liver recently which was confirmed by pathological examinations in a 18-year-old boy. He visited our hospital for examination of the progressive growing RUQ abdominal mass detected incidentally. On physical examination, a firm, non-tender, hard, fixed, huge, abdominal mass was palpated in the right upper quadrant of abdomen. Abdominal ultrasonography, computerized tomography and magnetic resonance imaging showed a 10x15cm sized mass confined to the left lobe of the liver, and it was hypovascular mass on angiography. We performed left lobectomy including the tumor and it was confirmed as undifferentiated (embryonal) sarcoma of the liver by pathologic examination. We herein report this case including histologic, electronmicroscopic and flow cytometric results with the review of literatures.
Abdomen ; Adolescent ; Angiography ; Chemotherapy, Adjuvant ; Humans ; Liver* ; Magnetic Resonance Imaging ; Male ; Mesenchymoma ; Physical Examination ; Prognosis ; Radiotherapy ; Rhabdomyosarcoma, Embryonal ; Sarcoma* ; Ultrasonography

BACKGROUND: Long term patency of arterial graft has been better than venous graft and redo coronary artery bypass grafting has been increasing, therefore, there has been an increasing need for alternative arterial grafts except internal thoracic artery(ITA). MATERIAL AND METHOD: Right gastroepiploic arteries(RGEA) were harvested from 100 patients who had received gastrectomy for gastric cancer or ulcer. ITAs were obtained from 10 patients undergoing coronary artery bypass grafting. The length of RGEA was measured from the pyloric ring. Items of the morphometric and histologic study at the pyloric ring and sites of the 10cm and 20cm RGEA from the pyloric ring were luminal diameter, intimal thickness, medial thickness, wall thickness, degree of intimal hyperplasia, intimal thickness, medial thickness, wall thickness, degree of intimal hyperplasia, intimal thickness index, medial thickness index, and the number of discontinuities of the internal elastic lamina. Similar items were applied to the proximal site of ITAs. RESULT: The length of RGEA was 23+/-2.7cm(range 17~31cm). Comparing the 20cm RGEA with ITA, intimal thickness, medial thickness, wall thickness, and degree of intimal hyperplasia did not show any difference(p>0.05). However, 20cm RGEA was greater than ITA at the luminal diameter, intimal thickness index, and the number thickness and wall thickness in each site of the RGEA(pyloric ring, 10cm, 20cm) decreased from the pyloric ring to the distal sites(p<0.05). The degree of intimal hyperplasia and the number of discontinuities of the internal elastic lamina did not show any difference between the pyloric ring and 10cm, however, those of 20cm were smaller than these sites(p<0.05). RGEA had more number of discontinuities of the internal elastic lamina and rich smooth muscle cells in the media than ITA. CONCLUSION: The length and diameter of RGEA is good enough to reach most of the coronary arteries. Moreover, long term patency of RGEA may be improved, if anastomosed in the distal site.
Coronary Artery Bypass* ; Coronary Vessels* ; Gastrectomy ; Gastroepiploic Artery* ; Humans ; Hyperplasia ; Myocytes, Smooth Muscle ; Phenobarbital ; Stomach Neoplasms ; Transplants ; Ulcer

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

PURPOSE: To evaluate 12-month outcomes of anti-vascular endothelial growth factor (VEGF) therapy for polypoidal choroidal vasculopathy (PCV) with grape-like polyp clusters. METHODS: This retrospective observational study included 23 eyes of 23 patients who were newly diagnosed with PCV with grape-like polyp clusters, and who were subsequently treated with anti-VEGF monotherapy. The study compares the best-corrected visual acuity (BCVA) of the patients at diagnosis, at 3 months, and at 12 months after diagnosis. In addition, 12-month changes in BCVA values were compared between cases with subfoveal or juxtafoveal polyps and cases with extrafoveal polyps. RESULTS: The baseline, 3-month, and 12-month logarithm of the minimal angle of resolution BCVA was 0.62 ± 0.35, 0.50 ± 0.43, and 0.58 ± 0.48, respectively. Compared to the baseline, patient BCVA was not significantly different at 12 months after diagnosis (p = 0.764). Six eyes (26.1%) gained ≥0.2 logarithm of the minimal angle of resolution BCVA. In cases with subfoveal or juxtafoveal polyps, BCVA values at baseline and at 12 months after diagnosis were 0.66 ± 0.37 and 0.69 ± 0.53, respectively. In cases with extrafoveal polyps, the values were 0.54 ± 0.33 and 0.37 ± 0.31, respectively. Changes in BCVA values were significantly different between the two groups (p = 0.023). CONCLUSIONS: Although anti-VEGF therapy has favorable short-term efficacy for treating PCV with grape-like polyp clusters, long-term visual improvements are generally limited in the majority of afflicted eyes. The presence of subfoveal or juxtafoveal polyps may suggest unfavorable treatment outcomes.
Aged ; Angiogenesis Inhibitors/*administration & dosage ; Choroid/blood supply/*diagnostic imaging ; Choroidal Neovascularization/diagnosis/*drug therapy ; Female ; Fluorescein Angiography ; Follow-Up Studies ; Fundus Oculi ; Humans ; Intravitreal Injections ; Male ; Polyps/diagnosis/*drug therapy ; Retrospective Studies ; Tomography, Optical Coherence ; Vascular Endothelial Growth Factor A/*antagonists & inhibitors