Purpose : Proliferative chondrocytes and prehypertrophic chondrocytes secrete significant amounts of type II collagen in an extracellular matrix. In contrast, hypertrophic chondrocytes secrete type X collagen. In addition, fibroblast growth factors (FGFs) and fibroblast growth factor receptors (FGFRs) also appear to play an important role during differentiation. Accordingly, the current study identified and characterized the chondrocytes and FGFR mRNA expressed... |
Purpose : In order to investigate the role of endothelins in the cardiac development, the present study was designed to examine the effects of endothelin A receptor(ETAR) antagonist to the cellular proliferation and apoptosis in the neonatal rat heart. In addition, the expression of various regulatory genes in protein and mRNA levels by ETAR antagonist were examined. Methods : Neonatal... |
Purpose : This study aimed to demonstrate the possible pathogenesis of granulopoiesis in patients of Kawasaki disease(KD) using quantitative analysis of G-CSF, GM-CSF and their CSFr. Methods : The plasma levels of G-CSF, GM-CSF, G-CSFr and GM-CSFr were studied in 14 patients in the acute phase of KD; 13 children with normal peripheral white blood cell counts were used as the... |
Purpose : Granulocyte-colony stimulating factor(G-CSF) and granulocyte macrophage-colony stimulating factor(GM-CSF) are principal cytokines in granulopoiesis and their physiologic effects are mediated through binding to specific cell surface receptors. Although it is known that the level of serum G-CSF and GM-CSF, and presentation of the receptors are increased in infectious diseases, there have been no studies to find the correlation between... |
The most common form of genetic nephrogenic diabetes insipidus(NDI), a rare inherited disorder, is congenital and is transmitted in an X-linked recessive mode. It is refractory to the antidiuretic effect of normal to moderately increased levels of plasma arginine vasopressin(AVP) but, in some cases, may respond to high levels of the hormone or its analogue, deamino-D-arginine vasopressin(DDAVP). X-linked congenital NDI... |
Purpose : It is well known that the receptors for Fcγ moiety of IgG(FcγR) are instrumental in antibody-mediated clearance of microorganisms by granulocytes and monocytes, and immune regulation in lymphocytes. Furthermore, complement receptor-3(CR3) is also important in neutrophilic adhesion, diapedesis, and phagocytic functions. In an attempt to examine their roles in neonates, we compared the expressions of FcγRs and CR3... |
Purpose : The development of external genitalia in genetic male is dependent on the transcriptional regulatory activity of dihydrotestosterone(DHT)-androgen receptor complex in the genital skin. The abnormality of androgen receptor encompasses a wide range of phenotypes. We investigated the androgen receptor binding capacity of genetic males with ambiguous genitalia(grade was determined by Prader grade) for the availability as screening test. Methods : The binding capacity... |
Purpose : Children with idiopathic short stature(ISS) are classified on the basis of exclusion criteria. Short stature with normal or increased circulating growth hormone(GH) and low IGF-I levels indicates that partial growth hormone insensitivity(GHI) may play a role in ISS. The present study was performed to investigate whether partial GHI is observed in children with idiopathic short stature and whether... |
Apert syndrome is an uncommon congenital disorder characterized by malformation of the skull in association with symmetrical syndactyly of both hands and feet. This syndrome is autosomal dominant. The original description was presented by Apert in 1906. Since then more than 200 cases have been reported in the world. Recently, we experienced a case of newhorn male infant with congenital... |
Purpose : A majority of patients with neuronal migration disorders(NMDs) in cortical structures suffer from medically intractable epilepsy. The role of NMDs on seizure susceptibility or epileptogenecity has not been well documented. In the present study, we established an experimental model of NMDs in Sprague-Dawley rats by exposing fetal rats to external irradiation in order to demonstrate epileptogenic effect of NMDs lesions. Methods : Pregnant... |
Purpose : Growth hormone(GH) is a powerful inhibitor of lipoprotein lipase and is known to decrease fat cell mass. The lipolytic effect has more pronounced influence on visceral fat than subcutaneous fat. The effects of GH therapy on GH receptor in fat tissue are not clear. We investigated the changes in fat tissue and GH receptor mRNA in adipose tissue... |
Purpose : The mechanism of hypoxic damage is mainly intracellular influx of calcium ions through the glutamate ionotropic receptor. This study was performed to determine alterations in distribution and expression of kainate receptor subunits after 1 hour of moderate hypoxia in the newborn piglet brain, as in a condition of mild to moderate perinatal hypoxic-ischemic encephalopathy. Methods : Ten newborn piglets... |
Purpose : The mechanism of hypoxic damage is mainly intracellular influx of calcium ions through the glutamate ionotropic receptor(NMDA, AMPA/kainate). This study was performed to determine alterations in distribution and expression of AMPA receptor subunits after 1-hour of moderate hypoxia in the newborn piglet brain, in a state of mild to moderate perinatal hypoxic-ischemic encephalopathy. Methods : Ten newborn piglets were... |
Crouzon syndrome, an autosomal dominant disorder, has characteristic features of craniosynostosis, hypertelorism, exophthalmos, maxillary hypoplasia and relative mandibular prognathism. Mutations of fibroblast growth factor receptor 2(FGFR2) gene are associated with craniosynostotic conditions, such as Crouzon syndrome, Jackson-Weiss syndrome, Pfeiffer syndrome, Apert syndrome and Beare-Stevenson cutis gyrata. We found one child with common morphological features of Crouzon syndrome. Interestingly, she... |
Purpose : The immature neonatal immune system is thought to result in increased risk of infection. Receptors for the Fcmoiety of IgG(FcγR) are important in antibodymediated clearance of microbes by monocytes/macrophages and immune regulation in lymphocytes. As an approach to understanding their role in neonatal life, we have compared the constitutive expression of the three Fc receptors-FcγRI(CD64), FcγRII(CD32) and FcγRIII(CD16)-by neonatal and adult blood monocytes... |
Purpose : Excessive amounts of glutamate are released into the extracelluar space during hypoxia/ischemia and contribute to neuronal injury through overactivation of the NMDA receptors. It will be expected that the changes of the NMDA receptors to decrease the injury are developed as a kind of defense mechanism. The NMDA receptors are composed of NR1 and NR2, and there are four NR2 subunits; NR2A,... |
Childhood minimal change nephrotic syndrome (MCNS) is often associated with hyper-sensitivity reactions and considered to be caused by immune dysfunction. The elevated serum IgE levels and atopic symptoms have been frequently associated in these patients. The present therapy for MCNS mainly depends on corticosteroids, alkylating agents such as chlorambucil, cyclophosphamide and cyclosporin A (Cy-A). Howerver, frequent relapses and severe side effects... |
Kawasaki disease has been known as an acute febrile mucocutaneous lymph node syndrome since 1967. But its definite pathophysiology still remains unclear. In order to study the immunologic basis for immunologic abnormalities in this disease, we studied the level of soluble interleukin-2 receptor (sIL-2R), interleukin-6 (IL-6) and C-reactive protein (CRP) at the acute and convalescent phases of the disease, comparing... |
The authors analyzed the clinical manifestations, laboratory results, and relationship between the presence of antithyroid autoantibodies and response of short-term antithyroid medication in 20 children with Graves* disease who were diagosed at the Department of Pediatarics, Chungnam National Universtiy Hospital from March 1985 to February 1990. The results were as follows; 1) The ratio of male to female was 1:9. 2) The age distribution of patients ranged from... |