BACKGROUND AND OBJECTIVES: Idiopathic restrictive cardiomyopathy is a very rare and poorly recognized disease in children. This study is performed to describe the clinical course and to define potential predictors of outcome. MATERIAL AND METHOD: We reviewed the medical records and diagnostic studies of 11 consecutive patients during the period from Jan.1991 to Aug. 2000. RESULTS: The age at diagnosis was 1.2-13 years (median 7 years) and the duration of follow up was 3-90 months (median 3.6 years). All except one were symptomatic (dyspnea in ten, chest pain in four). The chest pain was associated with significant ST depression on both resting and exercise ECG, suggesting myocardial ischemia. Two had complete heart block as either initial or terminal event. Cardiac catheterization was done in nine ( mean pulmonary arterial wedge pressure 23+/-6mmHg, systolic pulmonary arterial pressure 47+/-14mmHg, mean right atrial pressure 11+/-9mmHg). Echocardiographic dimensional ratio of left atrium and aorta (LA/Ao) was 2.41+/-0.58. Mitral E/A inflow ratio was 2.72+/-1.42, E wave deceleration time was 93.6+/-44.2ms. During follow up, six died. The 2 year and 5 year cumulative survival rates were 54.5% and 18.8% respectively. The predictor for nonsurvivor were pulmonary venous congestion and LA/Ao >2.5(p<0.05). Verapamil was tried in 6 cases without favorable effect in all. CONCLUSION: Considerable numbers of restrictive cardiomyopathy have myocardial ischemia associated with ST depression and chest pain. The patients with pulmonary venous congestion and severe left atrial enlargement (LA/Ao>2.5) were at risk for death, requiring prompt definitive treatment such as cardiac transplantation.
Aorta ; Arterial Pressure ; Atrial Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Cardiomyopathy, Restrictive* ; Chest Pain ; Child* ; Deceleration ; Depression ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart Atria ; Heart Block ; Heart Transplantation ; Humans ; Hyperemia ; Medical Records ; Myocardial Ischemia ; Pulmonary Wedge Pressure ; Survival Rate ; Verapamil

No abstract available.
Ductus Arteriosus, Patent*

Reye syndrome is a rapidly progressive encephalopathy with hepatic dysfunction, which often begins several days after apparent recovery from a viral illness, especially varicella or influenza A or B. Salicylate use was identified as a major precipitating factor for the development of Reye syndrome. With the recommendation to avoid use of salicylates in children, Reye syndrome has virtually disappeared in recent years. We report a case of Reye syndrome in a 5-month-old infant who had been treated with intravenous immunoglobulin and aspirin under the diagnosis of Kawasaki disease, and showed symptoms of sudden onset of irritability, rigidity, decreased activity, vomiting, poor appetite, lethargy, liver dysfunction without jaundice, coagulopathy, and hyperammonemia.
Appetite ; Aspirin ; Chickenpox ; Child ; Humans ; Hyperammonemia ; Immunoglobulins ; Infant ; Influenza, Human ; Jaundice ; Lethargy ; Liver Diseases ; Mucocutaneous Lymph Node Syndrome ; Precipitating Factors ; Reye Syndrome ; Salicylates ; Vomiting

Purpose: To assess the prevalence of dry eye disease (DED) and ocular surface conditions in patients before cataract surgery at a tertiary hospital in South Korea. Methods: This retrospective study included 96 eyes of 96 patients scheduled for cataract surgery from March to June 2023. The primary metrics of DED, including the Symptom Assessment in Dry Eye (SANDE) score, tear film breakup time (TBUT), tear secretion (Schirmer test), and ocular staining score (OSS, Oxford grading), were investigated. The proportion of patients with DED was determined according to the Asia Dry Eye Society (ADES) and revised Korean DED guidelines. Results: The patients’ mean age was 65.9 ± 8.3 years, with a mean SANDE score of 55.6 ± 30.5. The mean TBUT, OSS, and tear secretion were 3.5 ± 1.2 seconds, 1.3 ± 0.8 points, and 7.0 ± 5.4 mm, respectively. Among the patients, 85.4% exhibited a SANDE score of 20 or higher, 99% had a TBUT of < 7 seconds, and 88.4% exhibited one or more points on the OSS. According to the ADES and revised South Korean guidelines, 79.2% and 86.5% of patients were diagnosed with DED, respectively. Furthermore, all 96 patients exhibited at least one abnormal dry eye sign, regardless of symptoms. Conclusions The prevalence of DED in patients before cataract surgery was > 80%, with all patients exhibiting at least one ocular surface abnormality. Therefore, identifying and managing ocular surface abnormalities before cataract surgery is imperative.

Purpose: To assess the prevalence of dry eye disease (DED) and ocular surface conditions in patients before cataract surgery at a tertiary hospital in South Korea. Methods: This retrospective study included 96 eyes of 96 patients scheduled for cataract surgery from March to June 2023. The primary metrics of DED, including the Symptom Assessment in Dry Eye (SANDE) score, tear film breakup time (TBUT), tear secretion (Schirmer test), and ocular staining score (OSS, Oxford grading), were investigated. The proportion of patients with DED was determined according to the Asia Dry Eye Society (ADES) and revised Korean DED guidelines. Results: The patients’ mean age was 65.9 ± 8.3 years, with a mean SANDE score of 55.6 ± 30.5. The mean TBUT, OSS, and tear secretion were 3.5 ± 1.2 seconds, 1.3 ± 0.8 points, and 7.0 ± 5.4 mm, respectively. Among the patients, 85.4% exhibited a SANDE score of 20 or higher, 99% had a TBUT of < 7 seconds, and 88.4% exhibited one or more points on the OSS. According to the ADES and revised South Korean guidelines, 79.2% and 86.5% of patients were diagnosed with DED, respectively. Furthermore, all 96 patients exhibited at least one abnormal dry eye sign, regardless of symptoms. Conclusions The prevalence of DED in patients before cataract surgery was > 80%, with all patients exhibiting at least one ocular surface abnormality. Therefore, identifying and managing ocular surface abnormalities before cataract surgery is imperative.

Purpose: To assess the prevalence of dry eye disease (DED) and ocular surface conditions in patients before cataract surgery at a tertiary hospital in South Korea. Methods: This retrospective study included 96 eyes of 96 patients scheduled for cataract surgery from March to June 2023. The primary metrics of DED, including the Symptom Assessment in Dry Eye (SANDE) score, tear film breakup time (TBUT), tear secretion (Schirmer test), and ocular staining score (OSS, Oxford grading), were investigated. The proportion of patients with DED was determined according to the Asia Dry Eye Society (ADES) and revised Korean DED guidelines. Results: The patients’ mean age was 65.9 ± 8.3 years, with a mean SANDE score of 55.6 ± 30.5. The mean TBUT, OSS, and tear secretion were 3.5 ± 1.2 seconds, 1.3 ± 0.8 points, and 7.0 ± 5.4 mm, respectively. Among the patients, 85.4% exhibited a SANDE score of 20 or higher, 99% had a TBUT of < 7 seconds, and 88.4% exhibited one or more points on the OSS. According to the ADES and revised South Korean guidelines, 79.2% and 86.5% of patients were diagnosed with DED, respectively. Furthermore, all 96 patients exhibited at least one abnormal dry eye sign, regardless of symptoms. Conclusions The prevalence of DED in patients before cataract surgery was > 80%, with all patients exhibiting at least one ocular surface abnormality. Therefore, identifying and managing ocular surface abnormalities before cataract surgery is imperative.

Purpose: To assess the prevalence of dry eye disease (DED) and ocular surface conditions in patients before cataract surgery at a tertiary hospital in South Korea. Methods: This retrospective study included 96 eyes of 96 patients scheduled for cataract surgery from March to June 2023. The primary metrics of DED, including the Symptom Assessment in Dry Eye (SANDE) score, tear film breakup time (TBUT), tear secretion (Schirmer test), and ocular staining score (OSS, Oxford grading), were investigated. The proportion of patients with DED was determined according to the Asia Dry Eye Society (ADES) and revised Korean DED guidelines. Results: The patients’ mean age was 65.9 ± 8.3 years, with a mean SANDE score of 55.6 ± 30.5. The mean TBUT, OSS, and tear secretion were 3.5 ± 1.2 seconds, 1.3 ± 0.8 points, and 7.0 ± 5.4 mm, respectively. Among the patients, 85.4% exhibited a SANDE score of 20 or higher, 99% had a TBUT of < 7 seconds, and 88.4% exhibited one or more points on the OSS. According to the ADES and revised South Korean guidelines, 79.2% and 86.5% of patients were diagnosed with DED, respectively. Furthermore, all 96 patients exhibited at least one abnormal dry eye sign, regardless of symptoms. Conclusions The prevalence of DED in patients before cataract surgery was > 80%, with all patients exhibiting at least one ocular surface abnormality. Therefore, identifying and managing ocular surface abnormalities before cataract surgery is imperative.

We reviewed 26 cases of cardiac rhabdomyoma in children which were diagnosed between 1986 and 1991 in Seoul National University Children's Hospital. Twenty-two cases were associated with tuberous sclerosis. Total tumor count was 58 and mean tumor count per patient was "daehakgyo".23. Their cardiac rhabdomyomas were diagnosed from fetal period through 12 years after birth. Median age was 9."uiwon" months. Fifteen patients were males and rest 11 patients were females. Cardiac symtoms and signs at initial diagnosis were present in 12 cases. They consisted of cardiac murmur in "hakgyo", arrhythmia in 8 and congestive heart failure in "daehakgyo" cases (one with murmur and the other with arrhythmia). Cardiomegaly was seen in 9 cases on chest X-ray and other "byeongwon" case showed abnormal left cardiac border. Electrocardiography and 24 hour Holter monitorings showed arrhythmia in 15 cases. Clinically significant arrhythmias were found in 7 cases on 24 hour Holter monitorings. They consisted of 1with frequent premature ventricular contractions, "daehakgyo uigwadaehak" with frequent premature atrial contractions, "daehakgyo" with atrial tachycardia and "daehakgyo uigwadaehak" with atrio-ventricular block ("daehakgyo" cases of atrio-ventricular block had other arrhythmias also). The locations of tumors were left ventricle in 21, interventricular septum in 21, right ventricle in 13 and atrium in "daehakgyo uigwadaehak" tumors. Mean tumor count per patient in fetal period and infancy was "daehakgyo".86 and it was statistically much more than that after infancy which was "byeongwon".5. Mean tumor area which was calculated on two dimensional echocardiography was larger in fetal period and infancy(128.9 +/- 38.2mm2)than that after infancy(47.8 +/- 11.2mm2). Mean relative tumor area to aortic root dimension was also significantly larger in fetal period and infancy than that after infancy ("daehakgyo".07vs 0.30). In tuberous sclerosis, mean mass area and mean relartive tumor area to aortic root dimension in fetal period and infancy was significantly larger than those in patients after infancy. Of 26 cases, tumorectomy was done in "daehakgyo uigwadaehak" cases due to hemodynamic obstruction and arrhythmia. One case died immediately after operation and the other "daehakgyo" cases are still alive with the follow up period of 30 and 44 months each. Of the 7 patients with significant arrhythmia on 24 hour Holter monitorings, pacemaker insertion was done in "byeongwon" case who received tumorectomy and other 1 case was treated with anti-arrhythmic drugs. All the other patients did not need anti-arrhythmic drugs or other management.
Arrhythmias, Cardiac ; Atrial Premature Complexes ; Cardiomegaly ; Child* ; Diagnosis ; Echocardiography ; Electrocardiography ; Female ; Follow-Up Studies ; Heart Failure ; Heart Murmurs ; Heart Ventricles ; Hemodynamics ; Humans ; Male ; Parturition ; Rhabdomyoma* ; Seoul ; Tachycardia ; Thorax ; Tuberous Sclerosis ; Ventricular Premature Complexes

No abstract available.
Child* ; Heart Neoplasms* ; Humans

PURPOSE: Isolated noncompaction of the ventricular myocardium(INVM) can present as heart failure or arrhythmias in a child. It is a rare disorder, characterized by prominent trabecular meshwork and deep intertrabecular recesses. We still know little about the diagnosis, symptoms, and clinical outcomes of INVM. METHODS: We included in our study 6 patients who showed ventricular noncompaction on echocardiography. Patients were diagnosed as INVM were excessively prominent trabeculations with deep intertrabecular recesses were found on echocardiography. Patients who had other complex heart lesions such as pulmonary atresia with intact ventricular septum in addition to ventricular noncompaction, were excluded. RESULTS: Age at presentation ranged from 1 day 7 years, with follow up being as long as 6 years. Symptoms at initial presentation were heart murmur, paroxysmal supraventricular tachycardia, cyanosis, feeding intolerance, ventricular tachycardia, and cardiomegaly at fetal screening. Prominent trabeculations and intertrabecular recesses were observed at left ventricular apex in all six patients. All patients were alive at last follow-up. One patient showed WPW syndrome on electrocardiography. Echocardiography revealed decreased systolic function in 4 patients, and decreased systolic and diastolic function in 1 patient. One patient is currently asymptomatic. CONCLUSION: Six patients were diagnosed with INVM with various symptoms at initial presentation. Echocardiography is the most important tool in the diagnosis of INVM due to its morphological characteristics. INVM can rarely be the cause of long term systolic dysfunction, and early detection by echocardiographic screening may be beneficial.
Arrhythmias, Cardiac ; Cardiomegaly ; Child ; Cyanosis ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart ; Heart Failure ; Heart Murmurs ; Humans ; Isolated Noncompaction of the Ventricular Myocardium* ; Mass Screening ; Pulmonary Atresia ; Tachycardia, Supraventricular ; Tachycardia, Ventricular ; Trabecular Meshwork ; Ventricular Septum ; Wolff-Parkinson-White Syndrome