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Purpose : Vitamin D dependent rickets(VDDR) is a rare, autosomal recessively transmitted disorder characterized by hypocalcemia, hypophosphatemia, increased alkaline phosphatase, secondary hyperparathyroidism and many other clinical features. Type Ⅰ VDDR arises from primary deficiency in the renal 1α-hydroxylase that produces 1,25(OH)2D3. So patients with type I VDDR require life long administration of vitamin D. Methods : There had been 6 children(4... |
Purpose : Because vascular access sites in neonates are limited, intravenous(IV) medications must often be mixed with maintenance fluids, including parenteral nutrient(PN) solutions. This study was done to determine whether IV medications commonly prescribed in the neonatal in- tensive care unit(NICU) are compatible with the two neonatal PN solutions. Methods : The compatibility of neonatal PN solutions and selected other drugs... |
Purpose : To determine whether C-reactive protein(CRP) can be used as a parameter to assess the safety of discontinuing antibiotic therapy and allows a shorter course of therapy in neonates treated for suspected bacterial infection. Methods : We have experienced 193 cases of suspected neonatal bacterial infection at Pusan Maryknoll Hospital. CRP levels were measured daily by immunonephelometry. Infants with initial... |
Purpose : With the implementation of measles vaccination programs, the number of patients with measles has decreased. However, epidemics still occur with high morbidity in infants less than 1 year of age. This fact calls for the establishment of optimal preventive measures against measles. The study was carried out to determine the effect of measles vaccination on an infant immunized... |
Purpose : We compared pathogen recovery rates by obtaining two blood cultures instead of one blood culture containing 1ml and collecting a larger volume, 1 to 3ml. Methods : Total of 750 blood specimens from 250 patients with fever, a temperature higher than 39℃ and suspected bacteremia were obtained. Each patient had two samples of blood, A(1ml) and B(4ml), obtained at... |
Purpose : Intractable diarrhea during infancy is one of the major causes of infant mortality. But, its etiology, clinical courses, or methods of treatment are not well known. Therefore, we conducted a clinical approach to intractable diarrhea during infancy. Methods : We have retrospectively evaluated clinical characteristics, laboratory findings, methods of treatment, days required for recovery, in 23 infants who were... |
Purpose : Developmental processes of atria in embryos or fetuses of the iv/iv mouse were studied to find the differences between right and left atrial chambers and to identify the characteristics of atrial isomerism in the abnormal laterality syndrome. Methods : Seventy-three embryos from SI/Col iv/iv mice(Jackson's laboratory, USA) or fetuses at the 10th-13th day were used. They were examined using... |
Purpose : Myoclonic epilepsy with ragged red fiber(MERRF) syndrome is a disease of the mitochondrial encephalomyopathies, characterized by progressive myoclonus(action), epilepsy, cerebellar ataxia, intention tremor, muscle weakness, progressive dementia, sensorineural hearing loss and optic atrophy. Its inheritance is maternally inherited mitochondrial mutation, and its pathologic finding is characterized by ragged red fibers(RRF). Biochemically its defects are diverse. This study was... |
Purpose : Leptin is a protein encoded by the ob gene that is expressed in adipocytes. It regulates eating behavior by the action to the satiety centers in the hypothalamus. In ob/ob mouse, adipocytes can not produce leptin because of a mutation in the ob gene which results in obesity. Whereas, leptin concentrations were elevated in obese adults, and leptin... |
Purpose : Leptin is the product of the ob gene secreted by adipocyte. This study investigated whether leptin concentrations were elevated in obese children and the relationship between leptin concentrations and gender, and age in Korean children. Methods : We measured serum concentrations of leptin in 124 subjects(10 to 18 years, 62 girls and 62 boys). Height, weight, obesity index, and... |
Purpose : There are many methods to interpret the skin prick test which has been commonly used to find out the causative allergen in allergic disease. Among them two are most frequently used. One is to measure the absolute size of wheal which is caused by the allergen and the other is to compare the size of wheal for allergen... |
Seoul National University College of Medicine, Seoul, Korea Pulmonary hemorrhage is a rare but possibly fatal complication of systemic lupus erythematosus(SLE). We report a case of massive pulmonary hemorrhage in a 14-year-old boy recently diagnosed as SLE. He developed massive pulmonary hemorrhage during the courses of i.v. methylprednisolone pulse therapy, and did not respond to i.v. cyclophosphamide. However, he rapidly... |
Autoimmune hepatitis is an inflammatory liver disease characterized histologically by a dense mononuclaear cell infiltration of the portal tract, serologically by the presence of non-organ and liver specific autoantibodies and increased concentrations of IgG in the absence of a known etiology. Two types of autoimmune hepatitis are classified in the peripheral blood of antinuclear antibody and/or in antismooth muscle antibody(ANA/ASMA)... |
Superior mesenteric artery aneurysm is very rare complication of Marfan syndrome, especially in children. A 11 years old male patient was admitted to the hospital because of fluctuating high fever and diagnosed as infective endocarditis and Marfan syndrome. During antibiotics therapy, fever was slowly decreased but abdominal pain was developed and pulsatile abdominal mass was palpable in the midline of... |
Systemic lupus erythematosus(SLE), an autoimmune disease with multisystemic involvement, has been reported to be associated with a number of gastrointestinal complications. But pancreatitis is an unusual complication of SLE, occuring in only 3-4% of lupus cohort. Multiple mechanisms contributing to pancreatitis associated with SLE have been demonstrated which include vasculitis, necrotizing pancreatitis, corticosteroid administration, and vascular thrombosis. We experienced a... |
The subacute necrotizing lymphadenitis, also known as Kikuchi-Fujimoto disease, is a benign, self-limited disorder of unknown cause. This rare disease is characterized by fever, lymphadenopathy, leukopenia, and elevated sedimentation rate. This disease preferentially affects young women. The diagnosis of this disease is confirmed by involved lymphnodes biopsy. Evidence for autoimmune dysfunction has been reported, but is usually lacking. According to... |
Fetal alcohol syndrome is characterized by distinctive facial appearance, prenatal onset growth deficiency, an increased frequency of development and mental retardation, cardiac anomaly and genitourinary anomaly. Complete abstinence during pregnancy is recommended, since alcohol consumption in each trimester has been associated with abnormalities. We experienced a case of fetal alcohol syndrome in a 11-month-old female infant. Her mother had drunk... |