A Case of Late Infantile Neuronal Ceroid Lipofuscinosis

Neuronal ceroid lipofuscinosis, which is also known as Batten-Bielschowsky disease, is a group of neuro degenerative disorders, associated with various progressive symptoms including seizures, dementia, visual loss and cerebral atrophy. We experienced a case of late infantile neuronal ceroid lipofuscinosis in a 6-year-old boy who had progressive myoclonic seizures, ataxia, rapid psychomotor deterioration and visual loss. Photic stimulation at 2 to 5 Hz elicited a discrete spike and wave discharges in the occipital region on an electroencephalogram. Magnetic resonance imaging of the brain showed generalized cerebral and cerebellar atrophy. An electron microscopic examination of the skin revealed characteristic curvilinear inclusion bodies. An optic fundoscopy revealed a devastated retina and severe optic atrophy. We report this case with the brief review of related literature.

Four Cases of Scrub Typhus Treated with Azithromycin in Children

Tsutsugamushi fever is a serious febrile disease outwardly similar to malaria and various arboviral infections. The endemic area is Asia-Pacific. This disease are generally associated with disturbed habitats favoring large populations of vector larval trombiculid mites. Therapy with tetracycline, chloramphenicol or ciprofloxacin is currently recommanded. Unfortunately these drugs is suboptimal for children and pregnant women. Recently, azithromycin has been proven to be effective in therapy of scrub typhus. There is no evidence that azithromycin causes adverse reaction to developing fetus or children. We report four cases of tsutsugamushi fever in children successfully treated with azithromycin. This is the first report describing clinical application of azithromycin to tsutsugamushi fever in Korean children.

Surgical Repair of Inferior Sinus Venosus Defect: A Report Four Cases / 대한흉부외과학회지

Inferior sinus venosus defect is a rare lesion in which there is a large interatrial communication adjacent to the atrial connection of the inferior caval vein. The defect is located posteriorly and inferiorly, outside the confines of the true atrial septum, and partial anomalous pulmonary venous connections are the rule. We underwent surgical repair in four patients with inferior sinus venosus defect and partial anomalous pulmonary venous return. There were three males and one female with an age range from four months to 25 years. A cross-sectional echocardiogram and cardiac catheterization had been performed preoperatively in all patients, but the correct diagnosis had been made in only one case. Surgical repair was indicated due to congestive heart failure, and one patient of 4-month-old needed urgent operation. The repair was accomplished by suturing a untreated autologous pericardial patch to the right of the pulmonary veins, so that the defect was closed and all the pulmonary venous blood was directed to the left atrium. The preoperative knowledge of the unusual anatomy allows the surgeon to repair the anomaly without difficulties. For the patients in whom interatrial communication and anomalous pulmonary venous return are suggested, surgeon has to pay careful attention to the anatomical landmarks to avoid incorrect placement of the patch.

3 Cases of Acute Death dueto Pneumomediastinum, Pneumothorax and Subcutaneous Emphysema as a Complication of Miliary Tuberculosis

Pneumomediastinum, pneumothorax and subcutaneous emphysema are uncommon in pediatric practice, but they may be rarely ocured in association with respiratory distress and excessive ressusciation in the neonatal period, and as a complication of bronchial asthma, measles in childhood. And also, there was a report of a few cases that developed to pneumomediastinum and subcutaneous emphysema caused by vomiting in patient of diabetic coma. It is rare so far in this country that above events are complicated by miliary tuberculosis, but they may lead the patient to fatal and lifethreatening conditions, even though we perform a considerable medical treatment for that. We presnted 3 cases of 6 month 20 days old female infant, and each 10 year and 11 year old boys, developed to pneumomediastinu, pneumothorax and subcutaneous emphysema, and suddenly expired during hospital treatment of miliary tuberculosis. And, we reviewed the literatures, too.