Multicystic encephalomalacia is the condition defined anatomically by the presence of multiple cavities in the great part of both cerebral hemispheres. The most common cause of the condition was regarded as the circulatory disturbance during the perinatal period. Also,neonatal asphyxia was the most important cause of the circulatory disturbance. But we experienced a case of multicystic encephalomalacia in a liveborn twin with a stillborn... |
Narcolepsy is a serious, lifelong, disabling disorder characterized by a tetrad of symptoms including excessive daytime sleepiness, cataplexy, hypnagogic hallucination, and sleep paralysis. We experienced a 14 year old boy with a narcolepsy diagnosed by typical clinical symptoms above mentioned and multiple sleep latency test. A brief review of the related literatures was also made. |
The cholelithiasis and common bile duct(CBD) stone are rare problems among children. Endoscopic retrograde cholangiopancretogram(ERCP) and endoscopic sphincterotomy are rarely commomly utilized in the treatment of children, primarily because there are fewerindications. ERCP is an established procedure for visualization of the biliary tract. Endoscopic sphincterotomy with stone extraction has been accepted as the treatment of choice for CBD stone removal in adults. However, the role... |
Isolated angiitis of the central nervous system is a rare clinicopathologic entity characterized by vasculitis restricted to the vessels of the central nervous system without other apparent systemic vasculitis. It manifests headache, higher cortical dysfunction, focal neurologic dysfunction and cranial nerve palsies. We experienced a case of isolated angiitis of the central nervous system in 6 year-old girl who was admitted... |
Brachyecphaly is a kind of craniosynostosis. Because of premature closure of the coronal suture, the skull is shorter in the anteroposterior diameter but is widened with a high vault and the occiput and borehead are flattened. Diabetes insipidus had been reported in oxycephaly. We have experienced a case of central diabetes insipidus associated with brachcephaly. A brief review of related literatures... |
To validate the diagnostic significance of red cell indices in non-anemic iron deficiency and iron deficiency anemia, complete blood count, serum iron, total iron binding capacity, and serum ferritin were measured in 208 middle school girls between 13 and 15 years age. We used Reciever Operating Characteristic (ROC) curve to compare the diagnostic significances of various red cell indices (MCV,... |
We have experienced a 14 year old female patient who had suffered from headache, dizziness, exertional dyspnea and chest pain during 6 months. She was diagnosed as primary pulmonary hypertension by ultrasonogram and cardiac catheterization. On the cardiac catheterization, there was elevated pulmonary artery pressure and normal pulmonary wedge pressure. We report this case with related literature review. |
Clinical observation was carried out for 147 patients with mucocutaneous lymph node syndrome(MCLS) who were admitted to the Department of Pediatrics, Wonju Cristian Hospital during the period from June, 1983 to June, 1992. A spinal tap was performed on 114 of these patients and the following results were obtained. 1) The incidence of aspetic meningitis in patients with MCLS was 52.6%.(60... |
Purpura fulminans is one of rare consumptive coagulopathy in children. The most common prediasposing conditions of purpura fulminans are infectious disease like streptococcal infection and chickenpox. This disease is characterized by ecchymotic lesions that are usually distributed symmetrically on the lower extremities and buttocks. These ecchymotic lesions undergo necrosis, unless there is effective treatment. We experienced 3 cases of purpura fulminans... |
The VATER syndrome is a group of congenital anomalies with a nonrandom tendency for concurrence. Defects include vertebral, anorectal malformation, tracheoesophageal fisutla with esophageal atresia, radial-limb, vascular, and renal abnormalities. The critical period of organogenesis is at or before the sixth or seventh week of gestation. We experienced one case of VATER syndrome in a 1 day old male neonate having... |
Acute disseminated encephalomyelitis(ADEM) is an acute inflammatory demyelinating disease of the central nervous system. It is thought to be an autoimmune mediated disorder which occurs usually after viral or bacterial infections, or after immunization. The clinical manifestations reflect sudden onset of the diffuse involvement of the brain, spinal cord and the meninges. We experienced two cases of acute disseminated encephalomyelitis. The... |
While lipomas are common in the rest of the rest of the body, intracranial lipomas are rare condition which are usually found in the midline of the brain. The majority of these lesions show few or even no symptoms and hardly ever produce alarming neurological defects. We experienced a case of corpus callosum lopoma in a 7 year old boy, who... |
This study was conducted to find out the significance of nucleated red blood cell (NRBC) in neonates, especially associated with acute or chronic perinatal asphyxia. So, we compared NRBC counts in various neonatal groups, such as normal newborn, small for gestational age (SGA), premature, acute perinatal asphyxia, & hyaline membrane disease (HMD). In the first day of life, we examined... |
Tuberculous meningitis is still relatively common infectious disease of central nervous system in korea. The prognosis of tuberculous meningitis is closely related to the state at which treatment if started. Adenosine deaminase (ADA) is an enzyme which irreversibly hydrolyzes adenosine into inosine and ammonia. Recent investigations have suggested that the measurement of ADA activities in cerebrospinal fluid is useful in the... |
Brain computed tomograpy is particularly accurate in defining cerebrospinal fluid (CSF) space because of the relatively great density difference between CSF and brain parenchyma. This noninvasive procedure has very quickly become definitive in the diagnosis of hydrocephalus. However, while the typical clinical and radiological picture of a hydrocephalic patient leaves no doubt as to the need for adequate surgical treatment.... |
The double compartment hydrocephalus is now recognized as a clinical entity needing early diagnosis and treatment. By definition, the concept of double compartment hydrocephalus indicates seperate and individually progressing, usually sequential, hydrocephalus of the supratentorial and infratentorial CSF ventricles. In most instances, this entity consists of supratentorial hydrocephalus of the third and lateral ventricles (as a result of aqueductal stenosis) that has been treated... |
Citrobacter although once regarded as nonpathogenic, has been responsible for many well- documented pathologic conditions, especially in compromised hosts. These include urinary tract, pulmonary and bone infections, gastroenteritis, gangrenous ulcer with septicemia, meningitis, and brain abscess. Citrobacter organisms have a mosaic of 0, K and H antigens that are found in the Enterobacter- iaceae. Cross reactions with antisera of other members of the Enterobacteriaceae suggest... |
The importance of establishing authentic normal platelet count, mean platelet volume and platelet distribution width in the newborn has been emphasized, but they have not been firmly established. We have presented values for platelet count, mean platelet volume and platelet distribution width in the venous blood of term, small for gestational age and premature neonates at postnatal day 1 and 7. The results were... |
Transverse myelopathy is characterized by a sudden onset of motor weakness and sensory loss on the distribution under the affected spinal cord level. And slso, autonomic nerve functions may be disturbed at the same time. The etiology is still unknown. But it is thought that numerous conditions and diseases are associat- ed with it. We experienced 3 cases of transverse myelopathy preceded by Korean hemorrhagic... |
Hypertrophic cardiomyopathy is a common genetically transmitted disorder characterized by asymmetric septal hypertrophy and systolic anterior motion of the mitral valve. We have recently experienced identical twin with the hypertrophic cardiomyopathy confirmed by M-mode and 2-D echocardiography. It is the first report of hypertrophic cardiomyopathy in identical twin in Korea. So presented this case with a review of referential literatures. |
Toxic epidermal necrolysis is a bullous erythematous disease characterized by scalding appearance of the skin. And , it is divided into drug induced, miscellaneous, idiopathic group by the etiology. We experienced a cases of Phenytoin induced toxic epidermal necrolysis. So, we presented the case and reviewed the literatures about it. |
Asphyxiating thoracic dystrophy, a rare form of bony dysplasia, is characterized by extreme constriction of thorax, short limbed dwarfism, abnormalities of pelvic bone in neonatal period, and progressive interstitial nephritis in childhood. The incidence of this syndrome has been estimated at 1 per 100,000 to 130,000 live births and more than 100 patients have been reported worldwide. Infants with asphyxiating thoracic dystrophy usually have... |
Esophageal duplication, a term used synonymously with enteric cyst, foregut cyst, gastroenteric cyst, enteric duplication, and etc, is a rare developmental anomaly which occures during differentia- tion of the larynx, trachea, and bronchi from the primitive foregut. They may cause dysphagia, regurgitation, pneumonia, hemoptysis, and occasionally produce alarming airway obstruction. We experienced a case with chief complaints of pale appearance and swallowing difficulty in... |
A rare form of congenital cystic lung disease, characterized by the presence of one or usually multiple interconnecting cyst, is called congenital cystic adenomatoid malformation of the lung. This disease almost invariably presents either in live premature or stillborn infants, and death in those infants bom alive usually occurs within a few hours of birth. Infants with congenital cystic adenomatoid malformation usually have tachypnea,... |
Three-step guided compliance training and movement suppression time-out technique were used to treat desruptive, self-injurious, and aggressive behaviors in an 8-years old tuberous sclerosis with severe mental retardation. In compliance training, the child’s compliance behavior to verval and/or gestural demand was reinforced with edible and/or social reinforcers and the noncomplied demand was forcefully carried out through physical prompting. Movement suppression time-out was enforced whenever any... |
We have experienced a case of massive thymic hyperplasia in 2 months old female with about 1 month’s history of upper respiratory tract infection and inspiratory stridor. The chest X-ray and chest CAT scan examination revealed superior mediastinal huge mass and so surgical resection and pathologic examination were perfermed because of clinical suspicion of pathologic mass. The pathologic finding of mass showed massive thymic... |
Dapsone syndrome is a rare hypersensitivity reaction to Dapsone. The reaction included fever, malaise, dermatitis jaundice with hepatic dysfunction, lymphadenopathy, and hemolytic anemia. We have experienced a case of Dapsone syndrome in a 10 years old male. This patients had taken D.D.S. with 50 mg per day for 1 month to treat an unknown skin disease. After then, above mentioned symptoms and signs were... |
We experienced a case of truncus arteriousus associated with interrupted aortic arch, ventricular septal defect and patent ductus arteriosus. A 5 month old boy had complained of rapid respiration and feeding difficulty since birth. There was no visible cyanosis or clubbing toes and nails. There was a grade 2 〜3 ejection systolic murmur with maximum intensity at the left lower sternal border. Diagnosis was... |