No abstract available.
Arteries*

PURPOSE: The recurrence of Kawasaki disease has not been considered significant and has not been reported on literatures in Korea. Authors reviewed cases with recurrent Kawasaki disease to get informations about recurrent Kawasaki disease and to know whether there is any factor, if present, that can predict recurrence. METHODS: We reviewed and analyzed the hospital records of patients with recurrent Kawasaki disease who had been admitted to Inha University Hospital from January 1986 through December 1994. RESULTS: The total number of cases with Kawasaki disease was 266 during that period in Inha University Hospital. Seven patients were diagnosed as having recurrent Kawasaki disease, but four of them fulfilled five or more items of the diagnostic criteria for Kawasaki disease. So the proportion of recurrent cases was 1.5%(4/259)(or 2.7% (7/259) ?). Sex ratio was M:F=3:1. The ages at the first episodes of illness were from 7 months to 3 years 2 months (median=24 months), and those of the second episodes were from 11 months to 6 years 3 months (median=4 years 3 months). The intervals between two episodes were from 4 months to 4 years 2 months (median=1 year 9 months). No special aspect could be found in the clinical and laboratory findings of primary cases, compared with other cases with kawasaki disease. The clinical manifestations and courses of recurred cases were not significantly different from those of primary cases, except one recurred case who developed coronary aneurysm. CONCLUSIONS: The recurrence rate of Kawasaki disease seems to be higher than 1.5%(or 2.7%) at least. The risk factors for recurrence could not be found, and there was no specific aspect in the clinical manifestations and courses of recurrent Kawasaki disease.
Coronary Aneurysm ; Hospital Records ; Humans ; Korea ; Mucocutaneous Lymph Node Syndrome* ; Recurrence ; Risk Factors ; Sex Ratio

Idiopathic myelofibrosis (IMF), which is characterized by marrow fibrosis, leukoerythroblastic anemia, teardrop poikilocytosis and splenomegaly due to extrumedullary hematopoiesis, has known to have no form of therapy. On the ground of the possibility of reversing collagen deposion in IMF using 1, 25dihydroxycholecalciferol [1, 25(OH)2D3], we report here our observations of 5 patients (M:F=1:4) with IMF before and after treatment with 0.5 microgram/day of alfacalcidol, precursor of 1, 25(OH)2D3. In 3 fo 5 patients the hemoglobin rose and in 4 of 5 the platelet count increased. Follow-up marrow examination revealed that marrow trephine reticulin fibrosis decreased according as the amelioration of clinical and laboratory findings. But these did not persist except one patient in spite of the sustained use of alfacalcidol. Our results suggest that alfacalcidol may have a therapeutic role in some patients with IMF. More extensive studies will be clarify the action of alfacalcidol in IMF.
Anemia, Myelophthisic ; Bone Marrow ; Child* ; Collagen ; Fibrosis ; Follow-Up Studies ; Hematopoiesis ; Humans ; Platelet Count ; Primary Myelofibrosis* ; Reticulin ; Splenomegaly

Pulmonary function test is one of the most important tools in the management of patients with respiratory tract diseases and allergic diseases. As with the difficulties in performing the test, it has been seldom used in pediatric field. But with the advent of simple, computerized tools, pediatric pulmonologist and allergist try to manage the patients using PFT. Still the normal predicted values are variable among the reports. So it is important to have normal predicted value in Korean children. From April to July 1992, pulmonary function test was performed in 965(male: 490, female: 475) primary school-aged children except <3 or >97 percentile of Korean-children Physical developemental standards with history of allergic diseases and respiratory tract diseases ay SungNam, KyongKiDo. We evaluated the predicted normal values of the FEV1.0, FVC and PEFR and logarithmic regression equation setting the predicted values by using the microspirometer of Micromedical Ltd. England that could that could be easily applied to children. 1) Predicted values of pulmonary function test items were generally higher in boys than those of girls. 2) Correlation coefficient to the parameters examined was the highest in height 0.78, then age 0.75, weight 0.70 and chest circumference 0.61.
Child* ; England ; Female ; Gyeonggi-do ; Humans ; Peak Expiratory Flow Rate ; Reference Values ; Respiratory Function Tests* ; Respiratory Tract Diseases ; Thorax

Burkitt's lymphoma is a distinct pathologic entity characterized as a diffuse undifferentiated malignant lymphoma of B-lymphocyte origin. We experienced a case of Burkitt's lymphoma with bilateral renal enlargement in which a 3year-old male patient was admitted with complaints of abdominal distention and facial edema. Laboratory examination revealed positivity for CD 19, CD 10, CD 20 and c-myc on flow cytometry, bilateral renal enlargement(10x12cm in longitudinal length) on abdominal ultrasonogram and CT, malignant lymphoma of Buritt's type with prominent nucleoli and cytospin of cerebrospinal fluid. Initially he showed tumor lysis syndrome and clinical stage D by Zigler(stage IV by Murphy) with CNS involvement. After initial management with hydration, urine alkalinization and allopurinol, combination chemotherapy had been applied with the craniospinal radiotherapy according to the CCG 503 II regimane, with achievement of complete remission. Thus we report a case of Burkitt's lymphoma with bilateral renal enlargement with a biref review of literatures.
Allopurinol ; B-Lymphocytes ; Burkitt Lymphoma* ; Cerebrospinal Fluid ; Drug Therapy, Combination ; Edema ; Flow Cytometry ; Humans ; Lymphoma ; Male ; Radiotherapy ; Tumor Lysis Syndrome ; Ultrasonography

PURPOSE: There are some disagreements about the optimal dosages of intravenous gammaglobulin(IVGG) and oral aspirin(ASA) in the treatment of Kawasaki disease. So authors performed a prospective clinical study to evaluate the efficacy of IVGG 1g/kg plus ASA 50mg/kg/day. METHODS: We treated 29 patients who were admitted to Inha University Hospital from June 1993 through May 1994 with IVGG 1g/kg plus ASA 50mg/kg/d. We compared the outcomes of above patients with those of two other groups of patients, group A and B in authors' previous study. Group A(20 patients) had been treated with IVGG 2g/kg plus ASA 50mg/kg/d and group B(19 patients) with IVGG 2g/kg plus ASA 100mg/kg/d. The outcomes had been similar in group A and B, which was published on this journal in 1995 (vol. 38:378-385). RESULTS: 1) Twenty five patients(86.2%; group C) were given only one dose of IVGG 1g/kg, and remaining 4 patients(13.8%; group D) were given two doses of IVGG 1g/kg because of persistent high fever. 2) The age and sex distributions, durations of fever before treatment, and durations of ASA therapy in group C were not significantly different from those in group A and B (p>0.05). 3) Laboratory findings on admission in group C were not significantly different from those in group A and B, except that the mean ESR was lower in group C than in group A and B (35.1+/-19.8 vs 55.5+/-5.95 & 50.2+/-11.4mm/hr; p<0.01, respectively). 4) The durations of fever after treatment in group C were not significantly different from those in group A and B (1.32+/-1.07 vs 2.65+/-3.28 & 1.74+/-1.52 days; p>0.05, respectively). 5) In group C, the mean hemoglobin concentration at the 3rd week of illness was higher than in group A (11.1+/-0.98 vs 10.1+/-1.24g/dl; p<0.05), the mean platelet count at the 2nd week of illness was lower than in group A (59.4+/-18.0x10(4) vs 73.6+/-19.0x10(4)/ l; p<0.05), and the mean ESRs at the 2nd and 3rd week of illness were lower than in group A (43.3+/-14.7 vs 54.0+/-9.16, 31.9+/-19.0 vs 47.7+/-13.0mm/hr; p<0.05, respectively). Other follow-up laboratory findings in group C were not significantly different from those in group A and B. 6) Echocardiography was done 2 and 4 weeks after onset of illness. Coronary arterial dilation was observed in four(4/25; 16%) and two(2/23; 8.7%) patients respectively in group C, and the proportions were not significantly different from those in group A(40% & 25%) and B(31.6% & 10.5%) (p>0.05, respectively). In follow-up examinations, coronary aneurysm was observed in only one(1/23; 4.3%) in group C, which was similar to group A(1/18; 5.5%) and B(1/19; 5.2%) (p>0.05, respectively). Giant aneurysm was not observed in any patients. 7) Four patients(group D) were given one more dose of IVGG 1g/kg because high fever persisted 48 hours after injection of the first dose of IVGG. Afterthen fever subsided within 1 to 7 days. Echocardiography revealed mild coronary arterial dilation in two patients initially, but follow-up examinations revealed no coronary aneurysm. CONCLUSIONS: The medium-dose combined regimen with IVGG 1g/kg plus ASA 50mg/kg/d seems to be more cost-effective than the high-dose regimen with IVGG 2g/kg plus ASA 50-100mg/kg/d. If high fever persists 48 hours or more after the first dose of IVGG 1g/kg, it is desirable to give one more dose of IVGG 1g/kg.
Aneurysm ; Aspirin* ; Coronary Aneurysm ; Echocardiography ; Fever ; Follow-Up Studies ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Platelet Count ; Prospective Studies ; Sex Distribution

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.
Bronchopneumonia ; Child ; Humans ; Incidence ; Infant* ; Medical Records ; Mycoplasma pneumoniae* ; Mycoplasma* ; Pharynx ; Pneumonia* ; Pneumonia, Mycoplasma* ; Reference Values ; Seasons

Miller-Fisher syndrome is an acute poly-neuropathy typically characterized by the triad of ophthalmoplegia, ataxia, and areflexia. Other poly-neuropathy diseases include Guillain-Barre syndrome and Bickerstaff brainstem encephalitis. However, not all cases of Miller-Fisher syndrome have the typical three symptoms. These diseases have been differentiated by clinical manifestations and anti-GQ1b IgG antibody testing. In Miller-Fisher syndrome, anti-GQ1b IgG antibody is present in over 95% of patients; however, the frequency is not so high in normal subjects or in those with other poly-neuropathy diseases. Infectious illnesses, such as respiratory diseases and gastrointestinal diseases, usually precede Miller-Fisher syndrome. In general, Miller-Fisher syndrome is a self-limiting illness; however, a few cases of Miller-Fisher syndrome progressing to respiratory failure have been reported, particularly in children. In this case, we report abnormal angiographic findings. We report a case of atypical Miller-Fisher syndrome with internal carotid artery stenosis treated with prednisolone.
Ataxia ; Brain Stem ; Carotid Artery, Internal ; Carotid Stenosis ; Child ; Encephalitis ; Gastrointestinal Diseases ; Guillain-Barre Syndrome ; Humans ; Immunoglobulin G ; Miller Fisher Syndrome ; Ophthalmoplegia ; Polyneuropathies ; Prednisolone ; Respiratory Insufficiency

Immotile cilia syndrome is an inherited disorder characterized by specific ultrastructural defects of cilia and associated impairment of ciliary motion and mucociliary clearance. Disorders of ciliary structure or function result in chronic sinopulmonary diseases manifested as chronic sinusitis, bronchitis, otitis media, nasal polyposis, and ultimately bronchiectasis. In addition, situs inversus, dextrocardia, and infertility can be associated with dysfunctional ciliary activity. We experienced a case of immotile cilia syndrome presenting with recurrent bronchitis, pneumonia, chronic sinusitis, otitis media, and bronchiectasis. She was diagnosed by lack of dynein inner arm on electron microscopy. Treatment included chest percussion, bronchodilators, antibiotics, and surgical intervention. She has been followed up at regular intervals. We report this case with related literatures.
Anti-Bacterial Agents ; Arm* ; Bronchiectasis ; Bronchitis ; Bronchodilator Agents ; Cilia ; Ciliary Motility Disorders* ; Dextrocardia ; Dyneins* ; Infertility ; Microscopy, Electron* ; Mucociliary Clearance ; Otitis Media ; Percussion ; Pneumonia ; Sinusitis ; Situs Inversus ; Thorax

Fixed drug eruption normally presents as single or multiple sharply demarcated erythematous lesions that recur at the same location upon re-exposure to the offending agent. When the acute inflammation subsides, it often leaves residual hyperpigmentation. Commonly implicated substances are phenolphthalein, barbiturates, sulfonamides, tetracyclines, salicylates, gold and pyrazolone derivatives. Despite frequent use of acetaminophen, drug eruptions, especially fixed drug eruptions, due to acetaminophen are extrernely rare. We report here a childhood case of fixed drug eruption caused by acetaminophen, which is extensively used as an over-the-counter drug, as well as in medical therapy.
Acetaminophen* ; Barbiturates ; Drug Eruptions* ; Hyperpigmentation ; Inflammation ; Phenolphthalein ; Salicylates ; Sulfonamides ; Tetracyclines