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Case Report
Effect of Cultured Thymic Epithelium Transplantation in a Patients with Severe Combined Immunodeficiency
Soo Kyung Yun, Byoung Ho Cha, Woo Seung Jeoun, Dong Soo Kim
Clin Exp Pediatr. 1992;35(10):1443-1448.   Published online October 15, 1992
Severe combined immunodeficiency disease is caused by a group of genetic disease characterized by abnormal function of T and B lymphocytes. The treatment of choice for severe combined immunodeficiency disease is a HLA mached bone marrow transplant, but transplantation of cultured thymus epithelium was used to treat severe combined immunodeficiency disease in case of that mached, related bone...
Acquired Persistent Cytomegalovirus Infection : An Association with Common Variable Immunodeficiency
Min Hyea Kim, Young Mi Hong, Sung Joo Lee, Je Geun Chi, Dong Soo Kim
Clin Exp Pediatr. 1992;35(9):1272-1279.   Published online September 15, 1992
Acquired persistent cytomegalovirus infection was diagnosed by anti CMV antibody and lymph node biopsy and persisted in a 3-year-old boy with recurrent episode of high fever, lymphadenopathy hepatosplenomegaly and pneumonia. Initial immunologic abnormalities including low serum IgG, high IgM decreased T4 and T4/T8 ratio finally progressed to immunologic paralysis which was compatible to common variable immunodeficiency. This is the first case which...
Neonatal Purpura Fulminans due to Homozygous Protein C Deficiency
Jae-Hong Park, Sung-Sub Sim, Soo-Young Kim, Hee-Joo Jeon, Chan-Yung Kim, Hong Keun Oh, Jin-Yeong Jeong
Clin Exp Pediatr. 1992;35(5):696-703.   Published online May 15, 1992
Homozygous protein C deficiency is a rare herediatary disorder of blood coagulation resulting in microvascular and venous thromboses, usually purpura fulminans, starting shortly after birth. Protein C serves to inactivate the active forms of factor V and VIII, and increase the rate of fibrin degradation. For confirmation of homozygous protein C deficiency, the infant should have undetectable protein...
Original Article
Hepaplastin Test for Screen of Vitamin K Deficiency in Term and Preterm Neonates
Eun Mi Kim, Gyung Og Yu, Dong Rak Choi, Chong Young Park, Hae Ran Lee, Choon Myung Ro
Clin Exp Pediatr. 1992;35(5):614-620.   Published online May 15, 1992
To screen vitamin K deficiency in term and preterm neonates, the authors conducted Hepaplastin test based on 40 term and 40 preterm neonates who were delivered at Han Gang Sacred Heart Hospital. Also we compared Hepaplastin activity of term neonates with that of preterm neonates. 0.5mg or 1mg of vitamin K1 was injected intramuscularly immediately after birth according to the...
Quantification of Human Urinary Growth Hormone and Its Clinical Significance in the Diagnosis of Growth Hormone Deficiency
Seung Ryul Lee, Ho Seong Kim, Duk Hi Kim, Chang Mi Kim
Clin Exp Pediatr. 1992;35(5):595-601.   Published online May 15, 1992
Twelve-hour nocturnal urine was collecte in 5 patients diagnosed of having complete GH deficiency, 6 of having partial GH deficiency and 7 persons as normal control group. The GH levels in urine collections were evaluated, and the results are as follows. 1) The GH level of normal control group was 16.76¡¾1.91ng/12hr, 59.00¡¾6.92ng/gCr, 16.24¡¾2.52ng/m2, 0.58¡¾0.12ng/kg. 2) The GH level of complete GH deficiency...
A case of type VI Ehlers-Danlos syndrome.
Ki Soo Pai, Young Mi Chung, Ran Namgung, Chul Lee, Dong Gwan Han
Clin Exp Pediatr. 1991;34(12):1717-1723.   Published online December 31, 1991
Ehlers-Danlos syndrome is an inherited hetergenous disorder of connective tissue with characteris- tic clinical manifestations. In this article, a case of Ehlers-Danlos syndrome is presented. A nine months old female was admitted due to developmental delay. On physical examination, she had soft velvety skin and corneal opacity. Both hip joints were positive for Barlow test. There was hyper- mobility of both knee and elbow...
Effect of yeast-derived methionyl recombinant growth hormone on growth hormone deficient dwarf.
Jong Jin Seo
Clin Exp Pediatr. 1991;34(9):1269-1275.   Published online September 30, 1991
Fifteen GH deficient dwarfs were treated with LBD-003, the first year-derived rhGH produced in Korea. The growth promoting effect of LBD-003 was excellent and similar to that of the pituitary extract GH or the previous imported rhGHs. The serum concentrations of IGF-1 at 6th and 12th month of treatment showed significant elevation (p< 0.005, p< 0.001 respectively), but no significant correlation was observed between the...
Clinical effects of yeast derived recombinant methionyl growth hormone in growth hormone deficiency.
Byung Churl Lee
Clin Exp Pediatr. 1991;34(3):334-339.   Published online March 31, 1991
Since the success of human growth hormone (hGH) synthesis by recombinant DNA technology, these GH products are used in the treatment of growth hormone deficiency. Recently, methionyl-GH has been produced in the yeast, Saccharomyces cerevisiae 2150 by the use of a recombinant method. In this paper, the clinical efficacy and. immunogenicity of this GH was studied during therapy of 1 year in 20 children...
Transient symptomatic zinc deficiency in a breast-fed, post term infant.
Jin Bok Hwang, Soon Young Song, Woo Hyun Kwon, Chang Ho Han, Hye Li Chung, Young Dae Kwon
Clin Exp Pediatr. 1991;34(1):101-106.   Published online January 31, 1991
Acrdermititis enteropathica is a rare autosomal recessive disorder of zinc absorption and its cardinal symptoms appear at the time of weaning from breast milk which has been referred to as most appropriate formula to prevent and treat this disease. However, recent reports presented Transient Symptomatic Zinc deficiency (TSZD) in infants fed breast milk whom they treated with transitory zinc supplement with no recurrence. We experienced...
Diagnostic Significance in Case with Growth Hormone Deficient Dwarfs.
Duk Hi Kim, Mi Jung Park, Yan Kyu Lee
Clin Exp Pediatr. 1990;33(12):1699-1704.   Published online December 31, 1990
As the scope for treatment with GH widens, it is of interest to evaluate those criteria that are used conventionally for diagnosis of GH deficiency. 11 patients with hypotha lamic-pituitay lesions and 11 patients with idiopathic GH deficiency underwent retesting GH secretion with insulin or arginine stimulation test. All 11 parients with organic GH deficiency remained GH deficient. Of the 11 retested patients with...
Whole Cow's milk Related iron Deficiency Anemia in Early Childhood.
In Sang Jeon, Heon Seok Han, Hyo Seop Ahn, Jin Q Kim
Clin Exp Pediatr. 1990;33(10):1374-1379.   Published online October 31, 1990
To study the relationship between large amount of whole cow’s milk (WCM) intake and iron deficiency anemia (IDA) in early childhood, we carried out the study on 9 children with IDA, who were fed WCM, about 1,000 ml over 3 months, experienced at the department of Pediatrics, Seoul National University Hospital, during 5 months from March 1989 to July 1989. The results were as...
Prevalence Study of Anemia among Urban and Rural Middle School Girl Students.
Jeong Ok Hah, Mi Hwa Kang, Jeung Ho Kim
Clin Exp Pediatr. 1990;33(8):1087-1096.   Published online August 31, 1990
This study was conducted to investigate the prevalence rate of anemia and iron deficiency in pubertal girls in urban and rural areas. Venous peripheral blood was sampled from 346 girls who were randomly selected from 1,900 students of one girls’ middle school in Taegu city and all of 311 girls attending two middle schools in rural area, one in Cheungdo county and, another...
Therapeutic Effect of Iron Deficiency Anemia.
Dong Suk Lee, Chang Hee Han, Kun Soo Lee
Clin Exp Pediatr. 1990;33(6):799-806.   Published online June 30, 1990
A prospective study was conducted to see the therapeutic effect of iron deficiency anemia on a different oral iron doses. Studied patients consisted of 48 children with iron deficiency anemia who were admitted to our Pediatric Department during January 1988 - September 1989. Three groups were consisted with Group A (element iron dose 3 mg/kg/d, 16 cases), Group B (3 mg/kg/d with vitamin C...
Glucose-6 Phosphate Dehydrogenase Deficiency.
Meen Jai Lee, Sang Eun Lee, Dong Whan Lee, Sang Jhoo Lee, Sang Chul Park
Clin Exp Pediatr. 1990;33(2):212-219.   Published online February 28, 1990
Glucose-6-phosphate dehydrogenase(G-6PD) deficiency is the most common disease-producing enzyme deficiency of human beings, but extremely rare in Korea. Four well-characterized clinical syndromes are recognized: acute, oxidative stress(drug or infection) induced hemolytic anemia; neonatal hyperbilirubinemia; favism and chronic nonspherocytic hemolytic anemia. We have experi- enced a case of G-6PD deficiency in child associated with chronic hemolytic anemia and exacerba- tions of hemolysis by infection and drug...
A Case of Patient with Common Variable Immunodeficiency.
Keun Cheon Kim, Chang Gee Kang, Chang Hyun Yang, Dong Soo Kim, Kir Young Kim
Clin Exp Pediatr. 1989;32(8):1123-1127.   Published online August 31, 1989
Common variable immunodeficiency, as defined by World Health Organization classification of primary immunodeficiency, is a heterogenous group of disorders with hypogammaglobulinemia, decreased ability to produced antibody following antigenic challenge, and increased incidence of infections. The disorder was often familial, but no mode of inheritance has been discerned. Clinical fingings are recurrent bacterial infections, particularly respiratory infection. Laboratory evaluations in common variable immunodeficiency demonstrate decrease in...
Severe Iron Deficiency Anemia as a Result of Congenital Hiatal Hernia.
H Y Lee, H G Ham, M K Namgoong, B K Lim, J S Kim
Clin Exp Pediatr. 1989;32(2):250-254.   Published online February 28, 1989
Congenital diaphragmatic anomaly is a rare congenital malformation. A 6 year old boy with pale appearance and complaining of weakness for several months was diagnosed as having iron deficiency anemia as a result of congenital hiatal hernia. The diagnosis was confirmed by chest X.ray, esophagogram, upper gastrointestinal series and bone maπow biopsy After the hernia was successfully corrected by operation, the anemia was disappeared. Congenital...
A Study on Red Cell Distribution Width of Iron Deficiency Anemia in Childhood.
Hong Ryang Kil, Young Hun Chung
Clin Exp Pediatr. 1988;31(10):1321-1327.   Published online October 31, 1988
A hematologic study was undertaken on 60 cases of control group and 53 cases of iron deficiency anemia group who visited the outpatient department of Pediatrics, Chungnam National University Hospital from January, 1984 to July, 1987 The results were as follows: 1) In 60 cases of control group between 1 to 15 year, hemoglobin was 12.5±1.7 gm/dl, MCV 82.4± 3.8 fl, and RDW 13.1 ±1.1% and...
A Case of Iron Deficiency Anemia with Hypoproteinemia.
Seong Ho Kim, Woo Kill Lee
Clin Exp Pediatr. 1988;31(8):1054-1058.   Published online August 31, 1988
Severe iron deficiency anemia is occasionally accompanied by hypoproteinemia and edema. There is evidence that the hypoproteinemia is caused by increased intestinal loss of serum protein. We experienced a case, 1 year and 6 months old female patient with generalized edema, diagnosed as iron deficiency anemia with hypoproteinemia. After treatment with ferrous iron preparation, generalized edema was subsided, and hemoglobin value and serum protein...
Iron Deficiency Anemia in Childhood and Diagnostic Significance of Serum Ferritin.
Dong Hyub Lee, Ki Hong Park, Kyu Chul Choi, Yong Mook Choi
Clin Exp Pediatr. 1988;31(5):577-583.   Published online May 31, 1988
In order to elucidate clinical and laboratory findings of the iron deficiency anemia(IDA) in childhood along with diagnostic significance of serum ferritin, 207 patients were analyzed retrospec- tively, who were hospitalized at Department of Pediatrics, Kyung Hee University Hospital from January 1981 to December 1986. The results were as follows; 1) The incidence of IDA in In-Patient was 1.6%. 2) The sex ratio of male to female was...
6 Cases of Intracranial Hemorrhage due to late Late Hemorrhage Disease of Infancy.
Sang Ho Choi, Jae You Choi, Jee Sung Kim, Im Ju Kang
Clin Exp Pediatr. 1988;31(1):106-112.   Published online January 31, 1988
A bleeding syndrome in early infancy due to vitamin K deficiency has been reported sporadically from various parts of world. The syndrome has similarities to hemorrhagic disease of newborn. The main differences are that it is found in an older age group, the pathogenesis is unknown, and intracranial hemorrhage are frequent with a resultant high mortality rate. We have experienced 6 cases of spontaneous...
Case Report
A Case of Congenital Factor V Deficiency.
Byung Gug Jung, Hae Sung Cho, Jin Hyun Park, Jang Kwon Yang, Heui Jeong Kwon, In Sil Lee
Clin Exp Pediatr. 1987;30(9):1029-1033.   Published online September 30, 1987
Congenital factor V deficiency is a rare hemorrhagic disease characterized by the various hemorr- hagic manifestations and the prologations of clotting time, prothrombin time and partial thromboplastin time. We experienced a case of congenital factor V deficiency in a 4 month-old male infant, who had suffered from the bleeding tendncy since his neonatal period. We review literatures briefly and present this case.
Cranial Ultrasonographic Diagnosis for A Case of intracranial Hemorrhage in Early Infancy due to Vitamin K Deficiency.
Chul Lee, Ki Keun Oh
Clin Exp Pediatr. 1986;29(12):1377-1381.   Published online December 31, 1986
Since the initiation of routine vitamin K prophylaxis in the newborn, the incidence of hemorrhagic disease of the newborn has been dramatically decreased. We report here a case of intracranial hemorrhage due to vitamin K deficiency in a 2-month-old which was diagnosed with cranial ultrasonography. With advent of cranial ultrasonography which is portable, relatively inexpensive and noninvasive, the intracranial hemorrhage is easily confirmed especially as...
A case of Congenital Factor X III Deficiency.
Sei Woo Chung, Myung Ik Lee, Don Hee Ahn, Keun Chan Sohn, Jin Sook Hong, Dong Hun Yoon
Clin Exp Pediatr. 1986;29(5):553-559.   Published online May 31, 1986
A 4 year and 7 month old male patient was admitted with the chief complaint of subgaleal hematoma and recurrent bleeding episodes. The first episode was umbilical cord bleeding on the third day of life. Routine coagulation study including factor assay at that time was within normal limit. The bleeding cleared after whole blood transfusion. He had been admitted...
Three Cases of Familial Glucocorticoid Deficiency.
Soo Heum Lim, Young Wun Kim, Byoung Hai Ahn, Hyung Ro Moon
Clin Exp Pediatr. 1986;29(5):549-552.   Published online May 31, 1986
Familial glucocorticoid defiency is a form of chronic adrenal insufficiency which is characterized by isolated deficiency of glucocorticoid and elevated levels of corticotropin in association with normal aldosterone production. It is thought to be degenerative process of adrenal cortex, with autosomal recessive or X-linked recessive pattern of inheritance. Skin hyperpigmentation and hypoglycemic symptom are characteristic and salt-losing symptom doesn’t develop....
A Case of Congenital Nonspherocytic Hemolytic Anemia.
Tae Sook Kim, Sang Bae Jeon, Bok Yang Pyun
Clin Exp Pediatr. 1985;28(6):597-603.   Published online June 30, 1985
We observed a case of congenital nonspherocytic hemolytic anemia who was a 24 month old boy with the signs of jaundice 技 pallor. Evidences of the same disease was also found in the patient' s elder brother & father. We diagnosed these cases by history & physical examination, peripheral & B.M. blood smears, osmotic fragility test, autohemolysis test and etc. A...
Original Article
Primary Immunode Ficiency Disorders in Infancy and Childhood.
Hoan Jong Lee, Jung han Choi, Jeong Kee Seo, Hyo Sup Ahn, Kwang Wook Ko
Clin Exp Pediatr. 1985;28(3):263-282.   Published online March 31, 1985
The immunodeficiency disorders are a diverse group of illness which results from defectsin one or more of the four major mechanisms of immune system-antibody-mediated(B cell) immunity, cell-mediated(T cell) immunity, phagocytosis, and complements. Though immunodeficiency disorders are uncommon, the possibility should be considered in any individuals with recurrent or persistent infection. During the period between January, 1973 and October, 1984, we...
A study in The Changes of Platelet Count in iron Deficiency Anemia among Children.
Nam Ae Kim, Kir Young Kim
Clin Exp Pediatr. 1982;25(10):1015-1021.   Published online October 31, 1982
Iron deficiency anemia (IDA) is the most common nutritional anemia among chidren. A study on the changes of platelet count in IDA was conducted with 83 children who were diagnosed at the Severance Hospital in a 5 J/2-year pericd from Jan. 1975 to Jun. 1981. The results are summarized as follows. 1. IDA was most common among chidren younger than 2-years(61%) due to nutritional deficiency. It...
Incidenc of Iron Deficiency Anemia and Changes in Serum Feffitin Level in Various Childhood Diseases.
Hak Sunh Kim, Kwang Joo Rhee, Tae Won Kim
Clin Exp Pediatr. 1982;25(8):800-806.   Published online August 31, 1982
A serum ferritin level is now the most sensitive and specific assay for iron deficiency. Incidence of iron deficiency anemia was determined by means of the serum ferritin level and the response to iron therapy, and changes in serum ferritin level were observed in 421 hospitalized pediatric patients with various diseases. The results were as follows : 1. Incidence of...
Late Hemorrhagic Disease of Infancy due to Vitamin K Dependent Factor Deficiency.
Kang Il Lee, Yong Soon Kim, Sang Bum Lee, Ja Hoon Koo
Clin Exp Pediatr. 1982;25(1):52-57.   Published online January 31, 1982
A clinical study was done on 9 children with 10 episodes, who were admitted to our pediatric ward under the diagnosis of late hemorrhagic disease of infancy due to vitamin K dependent factor deficiency. Study period was past 2 years, from September 1979 to May 1981. The following results were obtained. This disorder occurred most frequently during early infancy, with...
Clinical Obseervation for Hematologic Disorders in Children.
Eung Sang Choi, Soon Ung Kang, Jeong Kee Seo, Hyo Seop Ahn, Chang Yoo Hong
Clin Exp Pediatr. 1981;24(3):235-244.   Published online March 15, 1981
Nine hundred and twenty-three cases of hematologic disorders in children who were admi-tted to department of pediatrics, SNUH, from 1955 to Oct. 1980 were analysed. The patients consisted of 624 males and 299 females. The results were as follows; 1.Mean annual percentage of hematologic patients among total admitted patients was 6.8%. 2.The peak age incidence of various hematologic disorders was...
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