Persistent Left Superior Vena Cava with Absent Right Superior Vena Cava and Large Atrial Septal Defect in Visceroatrial Situs solitus

Abscence of right superior vena cava(SVC) in visceroatrial situs solitus is a rare(0.07% to 0.13%) congenital cardiovascular malformation, and little is known about the type and frequency of additional heart defects and arrhythmias. We reviewed previous publications and report the case of 8 month old male infant with absent right superior vena cava and persistent left superior vena cava and large secundum atrial septal defect, mild pulmonary valvular stenosis.

Clinical Manifestations of Mycoplasma pneumoniae Pneumonia in Infants / 소아알레르기및호흡기학회지

PURPOSE: M. pneumoniae is knwon as a common causative agents of respiratory infection in school children. But, it tends to occur in infants and younger children recently. This study was performed to evaluate the incidence and clinical manifestations of M. pneumoniae pneumonia in infants. METHOD: A total of 142 children(33 infants{Group I : Infants group; 0-2 years} and 109 children{Group II : Children group; 3-6 years}) was admitted with M. pneumoniae pneumonia from January through December 1998. We reviewed medical records and evaluated the incidence, clinical manifestations and laboratory findings. RESULT: Number of patients with M. pneumoniae pneumonia in 0 to 2 years of age(infants group) comprised 23.2% of the total in contrast to 57.1% in 3 to 6 years of age group and 19.7% in 7 to 12 years of age group. Seasonal distribution showed the highest frequency in autumn in infant and children groups(45.5% and 39.4% respectively). The most frequent symptom was cough(90.9% and 96.3%, respectively), followed by sputum(81.8% and 90.8%, respectively), fever(72.7% and 66.0%, respectively) and rhinorrhea(72.7% and 64.0%, respectively). The common physical findings on admission were crackle(84.8% and 80.7%, respectively), wheezing(30.3% and 18.3%, respectively) and throat injection(39.4% and 35.8%, respectively). WBC count was within normal range in both groups(90.9% and 89%, respectively) and CRP was lower than 0.8 mg/dL in 63.6% of infant group and 54.1% of children group. The most common radiologic finding was bronchopneumonia in both groups (47% and 38%, respectively). CONCLUSION: Number of the patients with Mycoplasma pneumoniae pneumonia in infants age from 0 to 2 years comprised 23.2% of the total. Clinical manifestations of Mycoplasma pneumonia in infants were similar to those of older children. We recommened Mycoplasma pneumoniae should be considerd as a causative agent even in infant with respiratory infection.

Postnatal Change of Left Ventricular Contractility and Volume in Healty Term Infants with Ductus Arteriosus

No abstract available.

Assessment of Myocardial Ischemia using Myocardial Perfusion Scan in Kawasaki Disease

PURPOSE: Myocardial scintigraphy has been reported to be useful in adults, but its usefulness in children is limited. This study was done to determine the feasibility and accuracy of 99mTc-MIBI SPECT(sestamibi , methoxy-isobutyl-isonitrarite myocardial single emirs sion computed tomography, Dp-SPECT) after dipyridamole infusion to detect coronary obstructive lesions in Kawasaki disease(KD). METHODS: Dp-SPECT was performed in 21 control(group 1), 8 with coronary aneurysm(group 2) and 16 with coronary obstructive lesion(group 3) in children with KD. The spatial distribution of Dp-SPECT was determined and analyzed visually. Group 2 and 3 underwent coronary angiogram within 3 months of Dp-SPECT. RESULTS: Chest pain occurred more frequently in group 2. No subject required supplemental oxygen or were complicated by myocardial ischemia. The specificity of Dp-SPECT for control subject was 95% & 62% if using a cut-off criterion of abnormality, utilizing moderate or mild perfusion defect, respectively. The false positive rate was high in anterior, apical and anteroseptal segments but not related to age. The abnormalities found on Dp-SPECT was observed in 25% of aneurysmal coronary arteries but not related to its size. The sensitivity, specificity, and accuracy of Dp-SPECT was 100%, 84.5% and 79% in coronary stenosis greater than 75%, respectively. CONCLUSION: The interpretation of Dp-SPECT should be careful in KD because of discrepancies between Dp-SPECT & angiography, But DP-SPECT is noninvasive and easily applicable. It monitors the occurrence and progression of coronary stenosis due to KD. Besides Dp-SPECT may be applicable after arterial switch operation or to myocardial sinusoid.

Congenital Heart Disease and Associated Extracardiac Anomalies in Autopsies

PURPOSE: In order to find out how frequently and what kind of extra-cardiac anomalies (CAs) is associated with congenital heart disease (CHD) in general and in each CAs, autopsies with congenital cardiovascular malformations were studied. METHODS: Autopsy files of Seoul National University Children's Hospital from 1991 to 1995 were reviewed to find cases of CHDs. Other extra-CAs were tabulated from the records of autopsy files. The study population was comprised of 156 cases (86 still birth or product of terminated pregnancy; 66 livebirth; 4 unknowns-whether still birth or livebirth). RESULTS: The sex ratio was 1.14:1 males to females. In still birth cases, gestational age ranged from 16 to 42 weeeks and in live birth cases, age ranged from 1 day to 1 year 7 months (mostly neonates). The ventricular septal defect (VSD) was the most common CA, followed by coarctation of aorta, tetralogy of Follot in this order of frequency. No extra-CA was found in 55 cases, 1 extra-CA in 54 cases, 2 extra-CAs in 35 cases, 3 or more extra-CAs in 12 cases. VSD was commonly associated with Edward syndrome and holoprosencephaly, tetralogy of Fallot with holoprosencephaly, and coarctation with cystic hygroma. The most commonly involved organ system was the central nervous system followed by the digestive system, facial anomaly, and so on. Chromosomal anomaly associated with CHDs was 24 cases. Common individual anomalies were cystic hygroma, Edward syndrome, holoprosencephaly, and so on. CONCLUSION: As the fetus and infants with CHD have high incidence of associated extra-CAs, collaborative works involving dysmorphologist, geneticist, pathologist, surgeons, and pediatric cardiologist are needed. For further studies of CA and extra-CAs, a systematic database andprecise medical records and interdepartmental collaboration are necessary.

A Case of Chronic Granulomatous Disease Which is Improved by Interferon- Therapy

Chronic granulomatous disease is a group of rare disorders characterized by a disease of oxidative metabolism of phagocytes with deficit of antimicrobial activity. Also, it has clinical features of chronic pyogenic infection, granuloma formation, hypergammaglobulinemia, anemia and leukocytosis.We experienced a case of chronic granulomatous disease with chronic recurrent infection, hypergammaglobulinemia and liver abscess. It was confirmed by modified NBT test. The result of patient's NBT test was less than 11%. The patient received subcutaneous injection of interferon-gamma, 200 million unit (0.1mg/m2 of body surface area per dose), on three consecutive days per week for 3 weeks and her symptom was improved. Then, we report a case of chronic granulomatous disease which is improved by interferon- therapy with brief review of literature.