The hyperimmunoglobulin E syndrome is a primary immunodificiency disorder characterized by recurrent staphylococcal infections and markedly elevated serum IgE level. Clinical features are coarse face and severe infections of the skin-furunculosis or chronically pruritic dermatitis and sinopulmonary tract infection from infancy by coagulase positive Staphylococcus aureus, or Candida albicans etc. The patients's serum IgE level is elevated but the basic immunologic pathogenesis not fully understood. We have experienced a case of hyperimmunoglobulin E syndrome in a 26/12-year-old who had suffered from recurrent staphylococcal pneumonias and abscesses and chronically pruritic dermatitis from 1 month of age with elevated serum IgE level. A brief review of the related literature is presented.
Abscess ; Candida albicans ; Coagulase ; Dermatitis ; Immunoglobulin E ; Pneumonia, Staphylococcal ; Staphylococcal Infections ; Staphylococcus aureus

Glycogen storage diseases(GSD) are inherited disorders affecting glycogen metabolism and type I GSD is due to the absence or deficiency of glucose-6-phosphatase(G6Pase) enzyme in the liver, kidney, and intestinal mucosa. The defect leads to inadequate hepatic conversion of G6P to glucose and thus make affected individuals susceptible to fasting hypoglycemia, and the accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form of GSD and clinically growth retardation may manifest of GSD itself rather than growth hormone deficiency(GHD), but we experienced a case of type I GSD with GHD in a 14-year-o1d male. The height was 125 cm, compatible with 50 th percentile of height of 8 years of age. He has doll-like face with fat cheek, relatively thin extremities, and metabolic acidosis, hyperuricemia, hypoglycemia, hyperlipidemia. GH stimulation test with clonidine and L-dopa revealed that the patient had decreased GH secretion. After laboratory work up including liver biopsy, he was diagnosed as type I GSD. Hypoglycemia was managed with frequent feeding with high starch diet(uncooked cornstarch). Metabolic acidosis and hyperuricemia were treated with sodium bicarbonate, allopurinol and probenecid. The patient is being followed at out-patient clinic with clinical improvement after of diet therapy and GH administration.
Acidosis ; Allopurinol ; Biopsy ; Cheek ; Clonidine ; Diet Therapy ; Extremities ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone* ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Levodopa ; Liver ; Male ; Metabolism ; Outpatients ; Probenecid ; Sodium Bicarbonate ; Starch

PURPOSE: To get the normal predicted value of airway resistance in Korean kindergarten children by using a flow interrupter technique which is simple and easy enough to be applicable even to young children. METHODS: Two hundred and fifty children (male 126, female 124) were enrolled from two kinderdargen in Inchon. Excluded children were out of normal range of Korean growth standard, without any history and/or on-going symptom and sign of airway diseases. One medical doctor, well trained to the Microlab 4000 (Micromedical Co., England, educated those children in advance and carried out all the procedure. the Rint (airway resistance by interrupter technique) was checked 5 times. After the exclusion of both the highest and the lowest values, we got the average of remaining 3 values as the Rint (in both inspiratory and expiratory phases). The mean, standard deviation, regression equation, correlation coefficient for each group of age, height, weight, and chest circumference in both sex were investigated. RESULTS: 1) Age ranged from 3 to 5 years. 3-year-old boys' Rint were 1.89+/-0.76 kPa.l(-1).s and 1.78+/-0.71 kPa.l(-1).s in inspiratory and expiratory phase, respectively. Those for girls were 1.94+/-0.73 kPa.l(-1).s and 1.72+/-0.73 kPa.l(-1).s. The Rint was decreased as the children got older. 2) Height ranged from 91 to 120 cm. Rint of boys from 96 to 100 cm in height were 1.86+/-0.70 kPa.l(-1).s and 1.54+/-0.49 kPa.l(-1).s in inspiratory and expiratory phase, respectively. Those for girls were 1.68+/-0.72 kPa.l(-1).s and 1.71+/-0.77 kPa.l(-1).s. The Rint was decreased as the children got taller. 3) Weight ranged from 13 to 27 kg. Rint of boys from 13 to 15 kg in weight were 1.49+/-0.76 kPa.l(-1).s and 1.55+/-1.00 kPa.l(-1).s in inspiratory and expiratory phase, respectively. Those for girls were 1.78+/-0.83 kPa.l(-1).s and 1.82+/-0.76 kPa.l(-1).s. The Rint was decreased as the children got heavier. 4) Chest circumference ranged from 46 to 70 cm. Rint of boys from 51 to 55 cm in chest circumference were 1.69+/-0.84 kPa.l(-1).s and 1.52+/-0.74 kPa.l(-1).s in inspiratory and expiratory phase, respectively. Those for girls were 1.63+/-0.81 kPa.l(-1).s and 1.52+/-0.74 kPa.l(-1).s. The Rint was decreased as the children's chest circumference was increased. 5) Regression equations and correlation coefficients were calculated by each group of age, height, weight, and chest circumference for both boys and girls and in both inspiratory and expiratory phases. The correlation coefficient with age was around 0.5, which was the highest among them. CONCLUSION: The normal predicted value of airway resistance by flow interrupter technique was measured. It can be used for evaluating lung condition, progression of lung disease, and the effectiveness of drugs in lung disease.
Airway Resistance* ; Child* ; Child, Preschool ; England ; Female ; Humans ; Incheon ; Lung ; Lung Diseases ; Reference Values ; Respiratory Function Tests ; Thorax

Daily low-dose inhaled corticosteroid in preschool children with asthma is recommended to prevent the development of asthma symptom and sign based on many asthma management guidelines. But the retarded growth after long-term steroid inhalation has been concerned. Recently the method of intermittent high-dose (suspension, 1,000 microg, 2 times a day for 7 days) budesonide inhalation when the initial phase to develop the asthma exacerbation was studied to compare the efficacy and safety with daily low-dose budesonide (500 microg) inhalation regimen in preschool children positive with modified asthma predictive index in recurrent wheezer during the study period with 52 weeks. There were no significant difference between two regimen with respect to the frequency of exacerbations, the time to the first exacerbation and to the second exacerbation, and the frequency of treatment failure. The total exposure to budesonide over the period was less in the intermittent high-dose regimen than in the daily low-dose regimen. There were no significant difference in change in height. But the change in height from baseline in intermittent high-dose regimen group was 8.01 cm and in daily low-dose was 7.76 cm. We need the more clinical studies in preschool children with persistent moderate and severe asthma and in various country and nations. It is suggested the intermittent high-dose budesonide inhalation regimen in preschool children with freqeunt wheezing may be a very good alternative to control the allergic inflammation and symptoms of asthma without concerning the adverse effect of steroid.
Asthma ; Budesonide ; Child ; Child, Preschool ; Humans ; Inflammation ; Inhalation ; Respiratory Sounds ; Treatment Failure

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

To clarify the exact role of endomyocardial biopsy in the diagnosis and monitoring of adriamycin-induced cardiotoxicity and to observe the actual relationship between pathologic changes and cardiac dysfunction, a cross-sectional clinical study was conducted. Echocardiography was used to evaluate cardiac dysfunction in 18 patients who had received chemotherapy including adriamycin(mean dose : 410mg/m2 of B.S.A.) without clinical evidence of congestive heart failure, and in 19 normal controls. Six patients receiving adriamycin underwent 7 transfemoral endomyocardial biopsy procedures, and the specimens were evaluated by light and electron microscopy for evidence of drug-related cardiotoxicity. Indexes of cardiac systolic function obtained by M-mode echocardiography(left ventricular dimension, excursion of interventricular septum and left ventricular posterior wall, shortening fraction and ejection fracton) did not show any statistically significant difference between patients who received adriamycin and normal controls. In transmitral flow-velocity curves recorded by Doppler echocardiography with a 2.25MHz probe, the patients showed less E peak velocity and decreased E/A ratio compared with normal controls, which suggests left ventricular diastolic dysfunction in the patients who received adriamycin. All the specimens of the endomyocardial biopsy showed significant pathologic changes of adriamycin indnced cardiotoxicity which was characterized by myofibrillar loss and vacuolization of the cytoplasm. In 2 specimens, pathologic grade was II, while 5 specimens showed pathologic changes of grade III and further chemotherapy with adriamycine was not done in thse 5 cases. From these results it is suggested that pathologic changes precede the clinical onset of congestive cardiomyopathy in the patients receiving adriamycin and left ventricular diastolic dysfunction occurrs before ejection fraction falls to subnormal levels. We conclude that sequential endomyocardial biopsy is absolutely indicated for exact diagnosis and monitoring of adrinamycin-induced cardiotoxicity to prevent the development of irreversible and often fatal cardiomyopathy.
Biopsy ; Cardiomyopathies ; Cardiomyopathy, Dilated ; Cytoplasm ; Diagnosis ; Doxorubicin* ; Drug Therapy ; Echocardiography ; Echocardiography, Doppler ; Heart Failure ; Humans ; Microscopy, Electron

Although basic mechanisms of atopic dermatitis remain largely speculative, many studies on pathogenesis suggest the importance of food and inhalent allergens. To evaluate the frequency of food and house dust mite hypersensitivity and differences in this frequency according to ages, we measured the levels of specific IgE antibodies to egg white, egg yolk, milk, soy, and house dust mites in 119 children with atopic dermatitis. The results showed that 53% of patients had positive RAST to any one kind of allergens. The frequency of food and house dust mite hypersensitivity were 34.5%, 30.3 %, respectively. Among allergens, house dust mites and egg white are the most prevalent allergens in all atopic dermatitis patients. The Prevalence of egg white is most common under the age of 2 years, but those of house dust mites are the dust mites are the highest in the ages of 5-12 years. In conclusion, we recommend an egg restriction diet in atopic dermatitis patients who are less than 2 years old when their symptoms do not improve with general skin care.
Allergens ; Antibodies ; Antigens, Dermatophagoides ; Child* ; Child, Preschool ; Dermatitis, Atopic* ; Diet ; Dust* ; Egg White ; Egg Yolk ; Humans ; Hypersensitivity ; Immunoglobulin E ; Mites ; Ovum ; Prevalence ; Pyroglyphidae* ; Skin Care ; Soy Milk

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

Chronic cough-defined as a cough that persists for more than 3 weeks-is one of the most common symptoms during childhood that requires evaluation of causes and appropriate management, because it can be very disturbing to daily activities at home and school. Besides asthma, postnasal drip syndrome, post infectious cough, chronic bronchitis, gastroesophageal reflux disease and congenital anomaly, psychogenic factors are known to be possible causes of chronic cough in children. "Habit cough" and "respiratory tic" are different names given to psychogenic coughs. Psychogenic cough is croupy, loud, and unresponsive to antitussives or bronchodilators. It becomes more noticeable to attention and disappears during sleep. Over 90% of cases of psychogenic cough have been reported in patients under 18 years of age and its diagnosis is often delayed due to the time consumed for exclusion of other underlying organic disorders and the recognition of psychogenic factors as an etiology. We report on the case of an 11-year-old boy who presented with chronic cough of a barking nature and was diagnosed as having psychogenic cough by characteristics and 24-hour monitoring of cough frequency and who was treated by psychological interview.
Antitussive Agents ; Asthma ; Bronchitis, Chronic ; Bronchodilator Agents ; Child ; Cough* ; Diagnosis ; Gastroesophageal Reflux ; Humans ; Interview, Psychological ; Male

BACKGROUND AND OBJECTIVES: The therapeutic duration of variant angina is controversial. This study sought to determine the remission rates for coronary artery spasms, the factors associated with remission and the changes in spasm sites. SUBJECTS AND METHODS: Fifty-eight patients were enrolled in the study. Initial, and follow-up, coronary angiographies (CAG), with ergonovine stimulation tests, were performed. Paired CAG were performed at a mean interval of 27+/-17 months. Medication was stopped 3 days prior to the follow-up CAG, and the occurrence of chest pain during these 3 days was studied. Coronary spasms were confirmed by follow-up CAG. Any changes, and the diameters, of spasm sites were analyzed on each paired CAG. RESULTS: The remission rate of coronary spasms was 24% (14 patients), when the smoking group (49 patients) stopped smoking (31 patients), the remission rate was 29% (9 patients). In the current smoking group (18 patients), the remission rate was 6% (1 patient, p=0.05). 31 patients had chest pains after stopping medication prior to their follow-up CAG. Of those patients, 1 patient had a remission (3%). Among another 27 patients with no chest pain, 13 patients had a remission (48%, p<0.001). In 28 out of 44 patients (64%, non-remission), fluctuations in spastic locations were observed at the follow-up CAG. The interval changes in the diameter of the spasm sites were not significant. CONCLUSION: The non-chest pain group showed higher remission rates, but lack of chest pain did not identify the loss of coronary spasm. Atherosclerosis at spasm sites did not progress, as confirmed by the paired CAG in our study.
Angina Pectoris, Variant ; Atherosclerosis ; Chest Pain ; Coronary Angiography* ; Coronary Artery Disease ; Coronary Vessels ; Ergonovine ; Follow-Up Studies* ; Humans ; Muscle Spasticity ; Smoke ; Smoking ; Spasm*