ABSTRACT
X-linked agammaglobulinemia(XLA) is characterized by markedly reduced number of B lymphocytes, panhypogammaglobulinemia, recurrent bacterial infections in the first few years of life because of genetic defect for Bruton's tyrosine kinase at Xq22 region. Although XLA is a typical humoral immunodeficiency disease, severe neutropenia is sometimes presented in acute infection phase. We report a 23-month-boy with XLA who presented prolonged pneumonia, severe neutropenia over one month and profound panhypogammaglobulinemia. As his pneumonia improved, neutropenia subsided, but panhypogammaglobulinemia sustained. He was confirmed to have a point mutation in Btk-gene by direct-sequencing of Btk-gene.
Subject(s)
Agammaglobulinemia , B-Lymphocytes , Bacterial Infections , Neutropenia , Pneumonia , Point Mutation , Protein-Tyrosine KinasesABSTRACT
BACKGROUND AND OBJECTIVE: Asthma is the most common chronic disease among children. Thus, when children have asthma, it can have a significant impact on health-related quality of life, not only for children, but also for parents and family. We developed to measure asthma- specific quality of life in caregivers of children with asthma age 7~17 years and evaluated the measurement properties of the questionnaire. MATERIALS AND METHOD: The questionnaire of caregivers of children with asthma include 13 items in two areas (activity limitation-4 items, emotional function-9 items) and the response options for each item are on a 5-point scale where 1 indicates maximum impairement and 5 indicates no impairement. Results are expressed as the mean score for overall quality of life as well as for each of the domains. At follow-up visit, we asked caregivers whether they had experienced any change in their overall quality of life related to their children's asthma since the previous visit. They responded on a 11-point scale from -5 (a very great deal worse) to 0 (no change) to +5 (a very great deal better). We recruited 195 caregivers of children with asthma from thirty-three general hospitals and evaluated the measurement properties of the questionnaire. RESULT: The questionnaire of caregivers of children with asthma showed excellent responsiveness in changed group, but the change in score of stable and changed group has no difference. It was reproducible in subjects who are stable. There was no longitudinal and crosssectional correlations between caregiver's questionnaire score and the child's asthma status, while cross-sectional correlations were found between caregiver's questionnaire score and PEFR. CONCLUSION: The questionnaire of caregivers of children with asthma is simple and easy to use, and is applicable.
Subject(s)
Child , Humans , Asthma , Caregivers , Chronic Disease , Follow-Up Studies , Hospitals, General , Parents , Peak Expiratory Flow Rate , Quality of Life , Surveys and QuestionnairesABSTRACT
BACKGROUND AND OBJECT: Traditional asthma outcome measures such as symptoms, spirometry, and medication requirements usually provide valuable information about the asthmatic status but they rarely capture the functional impairments. Thus, many clinicians are increasingly recognizing the importance of an assessment of health-related quality of life (HRQL) as an outcome measure in asthma. We developed a questionnaire to measure asthma-specific quality of life in Korean children and evaluated the measurement properties. MATERIALS AND METHODS: The questionnaire for children with asthma included 14 items in three domains (symptoms-6 items, activity limitation-4 items, and emotional function-4 items) and the response options for each item were on a 5-point scale where 1 indicated maximum impairment and 5 indicated no impairment. Results were expressed as the mean score for overall quality of life as well as for each of the domains. At follow-up clinic visit, patients completed global assessment of change related to their symptoms, activities and emotions. We recruited children (7~17 years of age) with asthma from thirty-three general hospitals. RESULTS: Data from 214 patients (144 boys and 70 girls) who completed the questionnaire were analyzed. The mean age was 10.0 years and the disease severity consisted of mild intermittent (26.6%), mild persistent (36.0%), moderate persistent (32.2%), and severe persistent (3.3%). The questionnaire for children with asthma showed a high index of responsiveness, reproducibility, and both longitudinal and cross-sectional correlations with the conventional asthma indices and with the questionnaire score. But there was no relationship between changes in questionnaire score of the domain of emotional function and changes in FEV1 or PEFR. CONCLUSION: The questionnaire of children is straight forword, and easy to apply.
Subject(s)
Child , Humans , Ambulatory Care , Asthma , Follow-Up Studies , Hospitals, General , Outcome Assessment, Health Care , Peak Expiratory Flow Rate , Quality of Life , Spirometry , Surveys and QuestionnairesABSTRACT
PURPOSE: Childhood cancer is closely related to the mutation of tumor suppressor gene. The mutant gene may evoke congenital anomaly and development of cancer. The common solid tumors in childhood are Wilms' tumor, retinoblastoma and neuroblastoma. The cytogenetic study has been performed. The cytogentic study revealed structural abnormality of chromosome in Wilms' tumor and retinoblastoma. The oncogene and mutation of tumor suppressor gene are applicable to the study of carcinogenesis of childhood cancer. We studied to investigate the expression of p53, Rb and WT-1 in Wilms' tumor and retinoblastoma. METHODS: Immunohistochemical study has been performed to investigate the expression of p53, Rb and WT-1 in 7 cases of Wilms' tumor and 4 cases of retinoblastoma of childhood cancer. RESULTS: The positive reaction for p53, Rb and WT-l was seen in the nuclei of tumor cells. The positive reaction for tumor suppressor gene products in Wilms' tumor showed that p53 (mutant type) was 57.1%, Rb 85.7%, WT-l 28.6%. The immunohistochemistry for tumor suppressor gene products in retinoblastoma revealed that p53 protein (mutant type) was seen in all the cases, but Rb and WT-l protein were not seen. CONCLUSION: These results suggested that Wilms' tumor may be partially related with mutation of p53, Rb and WT-1, and retinoblastoma may have a relationship with mutation of p53 and Rb.
Subject(s)
Carcinogenesis , Cytogenetics , Genes, Tumor Suppressor , Immunohistochemistry , Neuroblastoma , Oncogenes , Retinoblastoma , Wilms TumorABSTRACT
PURPOSE: Analyzing of the causes of childhood death will provide us with the basis to understand prevalent diseases and to make the best possible health plan according to the studies. The National Statistical Office (NSO) of Korea has released an annual report of nationwide death. However, it could have underestimated the death toll of children because of delayed or missing reports of infants who died during the neonatal period. The death reports by pediatricians at hospitals could compensate for the dropped number of infantile death reported by the NSO. METHODS: We collected the death records for children under 15 years of age from the 136 secondary or tertiary referral hospitals over the country from January 1 through December 31, 1995. Then we tried to make a compilation of the records from both the NSO and our committee to finalize the cause of death. RESULTS: According to the records of the 136 hospitals, the total number of childhood death was 3,900 in 1995, including 1,790 deaths in the first 28 days of life. Most deaths occurred in the hospital (89.0%), which were confirmed either by a pediatrician (6.2%) or by a pediatric resident (58.8%). We were compiling 6,735 deaths according to the NSO and 3,415 of our own, with 846 overlaps. Seventeen deaths recorded by the NSO occurred in the first 6 days of life, compared to 1,292 from our own. After the first year, 4,762 deaths recorded by the NSO and 790 of ours were observed. CONCLUSION: The death records from hospitals could be enough to compensate for the dropped number of neonatal deaths recorded by the NSO, depicting more accurately the real pattern of death for Korean children in 1995.
Subject(s)
Child , Humans , Infant , Cause of Death , Death Certificates , Korea , Tertiary Care CentersABSTRACT
PURPOSE: Analyzing of the causes of childhood death will provide us with the basis to understand prevalent diseases and to make the best possible health plan according to the studies. The National Statistical Office (NSO) of Korea has released an annual report of nationwide death. However, it could have underestimated the death toll of children because of delayed or missing reports of infants who died during the neonatal period. The death reports by pediatricians at hospitals could compensate for the dropped number of infantile death reported by the NSO. METHODS: We collected the death records for children under 15 years of age from the 136 secondary or tertiary referral hospitals over the country from January 1 through December 31, 1995. Then we tried to make a compilation of the records from both the NSO and our committee to finalize the cause of death. RESULTS: According to the records of the 136 hospitals, the total number of childhood death was 3,900 in 1995, including 1,790 deaths in the first 28 days of life. Most deaths occurred in the hospital (89.0%), which were confirmed either by a pediatrician (6.2%) or by a pediatric resident (58.8%). We were compiling 6,735 deaths according to the NSO and 3,415 of our own, with 846 overlaps. Seventeen deaths recorded by the NSO occurred in the first 6 days of life, compared to 1,292 from our own. After the first year, 4,762 deaths recorded by the NSO and 790 of ours were observed. CONCLUSION: The death records from hospitals could be enough to compensate for the dropped number of neonatal deaths recorded by the NSO, depicting more accurately the real pattern of death for Korean children in 1995.
Subject(s)
Child , Humans , Infant , Cause of Death , Death Certificates , Korea , Tertiary Care CentersABSTRACT
OBJECTIVE: A Study was undertaken to assess the family background of abandonment and clinical and social aspects in an institute for foreign adoption. The results were compared to the previous two reports from the institute. METHOD: A sample of 1,728 children from 1987 to 1992 formed the subjects of the study by the medical records in the institution. RESULTS: 1) The unmber of girls were more than that of male and ratio of male to female was 1:1.1, which was reduced compared to previous study. 2) The distribution less than one month of age was most commonly and increased compared to previous study. 3) The Place at birth was hospital most commonly. 4) The type of delivery was full-term one(61.5%) and tend to be increased. 5) Age distribution of monthers was from 16 years to 25 years mainly, 1,081 of them(62.5%) were from unmarried one and 409(37.4%) were from married one. 6) By classifying the cause of adoption, unmarried mothers occupied main one(62.5%), poverty(13.4%) extramarital relations(5.2%), divorce(2.9%) and death of parents in the order of frequency, tend to increased in unmarried mothers. 7) Disease pattern showed respiratory disease, jaundice, acute gastroenteritis, pneumonia, urinary tract infection, conjunctivitis in order of frequency. By care of foster mother at private home since 1988, the incidence of the transmissible disease was reduced. 8) Congenital anomalies were cleft and palate, hydrocele, umbilical hernia and congenital heart disease in order frequency. 9) Three hundred and eighty two babies were admitted to general hospital. The main disease were bhaline membrane disease. Jaundice, pneumonia and urinary tract disease, sepsis in the order of frequency. CONCLUSIONS: The ratio of male to female in the institution showed no definite difference and the age was tend to be younger. The cause of adoption was unmarried mother mainly. Respiratory disease in the institution and hyaline membrane disease in the hospitalized was most common.
Subject(s)
Child , Female , Humans , Infant, Newborn , Male , Age Distribution , Child, Institutionalized , Conjunctivitis , Gastroenteritis , Heart Defects, Congenital , Hernia, Umbilical , Hospitals, General , Hyaline Membrane Disease , Illegitimacy , Incidence , Jaundice , Medical Records , Membranes , Mothers , Palate , Parents , Parturition , Pneumonia , Sepsis , Single Person , Urinary Tract Infections , Urologic DiseasesABSTRACT
During The last 11 years from Jan. 1980 to Dec. 1990, 72 cases of salmonellosis has been admitted to the Department of Pediatrics of National Medical Center and evaluated clinical difference between typhoid fever and salmonella gastroenteritis. The results were as follows: 1) The annual incidence of salmonella gastroenteritis insalmonellosis was increased in the latter half of the eighties. 2) Both salmonellosis had its peak incidence in the summer. 3) Salmonella gastroenteritis was highest in the age of less than 5 year in contrast with the age of 6~9 year in typhoid fever. 4) The specimens isolated salmonellae were blood and stool in most cases, especially blood in typhoid fever and stool in gastroenteritis. 5) S.typhi was 16 cases, salmonella group A was 3 cases, group B was 16 cases and Group D and E were a case in each in isolated salmonellae. 6) On the antibiotic sensitivity test, S.typhi was sensitive to the most antibiotics. but the salmonella group B was relatively resisitant to Ampicillin, Carbenicillin, Tetracycline and Chloramphenicol.
Subject(s)
Ampicillin , Anti-Bacterial Agents , Carbenicillin , Chloramphenicol , Gastroenteritis , Incidence , Pediatrics , Salmonella , Salmonella Infections , Tetracycline , Typhoid FeverABSTRACT
The clinico-pathological observation was done on 28 children with recurrent hematuria, who had been admitted to the Pediatric ward of NMC from January 1981 to July 1991. The results were as follows; 1) Most of the children with recurrent hematuria were over 6 years of age(24 cases, 85.7%)and the sex ratio was about 2.5:1. 2) IgA nephropathy (17 cases, 60.7%) was the leading pathologic lesion and mild focal nonspecific glomerulonephritis (5 cases), membranous nephropathy (2 cases), minimal chage (2 cases), Alport syndrome (1case), abnormal glomerular basement membrane (1 case) followed. 3) Types of recurrent hematuria ware gross in 22 cases and microscopic in 6 cases. Flank pain was noted only 1 case. The prodromal events of hematuria were upper respiratory infection in 15 cases and severe exercise in 2 cases. 4) There were no significant differences in clinical features and laboratory findings between IgA nephropathy and other pathologic lesions, except for higher frequency of proteinuria in former group. 5) During the follow-up periods, three cases were resulted in chronic renal failure and they were two cases of IgA nephropathy Class IV and one case of Alport syndrome. And one case of IgA nephropathy was resulted in nephrotic syndrom.
Subject(s)
Child , Humans , Flank Pain , Follow-Up Studies , Glomerular Basement Membrane , Glomerulonephritis , Glomerulonephritis, IGA , Glomerulonephritis, Membranous , Hematuria , Kidney Failure, Chronic , Nephritis, Hereditary , Proteinuria , Sex RatioABSTRACT
A 7 day old female baby suffered from perinatal asphyxia and trauma at birth. Subsequently, she had a violeceous subcutaneous plaque on back. The clinical setting, the skin manifestation and the histologic findings of the lesion were diagnostic of subcutaneous fat necrosis of the newborn. Histologic findings revealed fat necrosis with infiltration of inflammatory cells, foreign body giant cells and several fat cells contain needle shaped cleft in radial arrangement. Four weeks latter, the skin lesion was healed spontaneously.
Subject(s)
Female , Humans , Infant, Newborn , Adipocytes , Asphyxia , Fat Necrosis , Giant Cells, Foreign-Body , Necrosis , Needles , Parturition , Skin , Skin Manifestations , Subcutaneous FatABSTRACT
Cow milk allergy can be defined as an adverse immunologic reactions to cow milk protein. The term is often mistakenly applied to other causes of milk intolerence, such as lactase deficiency and galactosemia, which must be differenciated and excluded. We have experienced 10 children of cow milk allergy at neonatal onset who had suffered from G-I symtoms, such as diarrhea, irritability, weight loss, vomiting and abdominal distension. One half of this patients had family history of allergic diseas. All patients have positive specific IgE RAST to cow's milk protein, milk elimination test and milk challenge test. Brest milk feeding and soy bean formula feeding and soy bean formula feeding have started after diagnosis and then gastrointestinal symtoms and signs of all patients are improved but other allergic diseases are combined in 5 children.
Subject(s)
Child , Humans , Infant , Diagnosis , Diarrhea , Galactosemias , Hypersensitivity , Immunoglobulin E , Lactase , Milk , Milk Hypersensitivity , Milk Proteins , Glycine max , Vomiting , Weight LossABSTRACT
A clinical study was made on 85 cases of prematurity under 37 weeks of gestational age who had history of blood transfusion during their admission at the National Medical Center from January 1989 to June 1992. The results were as follows: 1) Among 339 prematurity patients, 85 patients(25.1%) received blood transfusion once at least. 2) Male to female ratio was 1.13:1 and mean gestational age was 32.5 2.7weeks (range:25~36 weeks). 3) Most of blood transfusion(66/85 cases) were performed within first week of life. 4) 67 patients(78.8%) received blood transfusion 3 times or less. 5) Combined diseases were hyaline membrane disease(69.4%), jaundice(51.8%), sepsis(30.6%), PDA (14.1%), DIC (8.2%), pulmonary hemorrhage(7.1%), intraventricular hemorrhage (7.1%), pneumonia(7.1%) and hepatitis(4.7%). 6) Clinical manifestations at the time of blood transfusion were pallor (47.1%), decreased activity (44.7%), bradycardia (23.5%),apnea (21.2%), tachypnea (20.0%), lethargy (4.7%), poor weight gain (3.5%) and tachycardia (3.5%). 7) The values of hematocrit were significantly increased after transfusion(42.4 +/-7.2 vs 34.6 +/- 6.8vol%). 8) The episodes of bradycardia were significantly decreased after blood transfusion(0.09 +/- 0.39 vs 0.23+/- 0.78)while the episodes of apnea were not (0.15 +/-0.71 vs 0.27+/- 0.84).
Subject(s)
Female , Humans , Male , Apnea , Blood Transfusion , Bradycardia , Dacarbazine , Gestational Age , Hematocrit , Hemorrhage , Hyalin , Lethargy , Membranes , Pallor , Tachycardia , Tachypnea , Weight GainABSTRACT
No abstract available.
Subject(s)
Mycoplasma pneumoniae , Mycoplasma , Pneumonia , Pneumonia, MycoplasmaABSTRACT
This report is a clinical analysis and comparative study with previous report of bronchial asthma at National Medical Center on bronchial asthma in children who were admitted to the MP dept. of national Medical Center from January 1987 to December 1990. The results of the study were as follows: 1) The out-break ratio of bronchial asthma is 3.7% of hospitalized children which is no change that about 4% since 1980. 2) The children under 6 years of ge was 62.7% which among under 3 years was 33.1%. The rate of under 3 year children is increased as previous study. 3) The out-break of bronchial asthma was most common in September and October and so peak incidence was noted in fall and duration of hospitalization was most 2 week. 4) Familial allergic history was positive in 54.6% and bronchial asthma was most common. The presence of allergic past history was 52.2%) and atopic dermatitis was most common 5) Eosinophilia was found in 80.8% and elevated IgE was also in 61.6% of bronchial asthma. 6) We could get positive results of IgE RAST and skin test: D. pteronyssinus, D. farinae, Houst dust. 7) The most frequent findigs of the chest radiograph were within normal limits. 8) The clinical improvement rate of bronchial asthma is 52.5%.