| Purpose : In this study, efforts have been made to evaluate the sella MRI in 14 patients with growth hormone deficiency to define more clearly the structural abnormalities of the hypothalamohypophyseal axis, examine frequency of these abnormalities, and determine whether a relationship exists between the sella MRI findings and types of endocrine dysfunction. Methods : Sella MRI was performed in 14 patients who were... |
| Wolman' disease is a rare autosomal recessive disorder in which a deficiency of lysosomal acid lipase leads to the accumulation of cholesteryl esters and trigycerides in most of the body tissues. Clinically, it is characterized by abdominal distention, hepatosplenomegaly, vomiting, intractable diarrgeam steatorrhea, malabsorption, inanitionm failure to thrive, and bilateral enlargement and calcification of the adrenal glands demonstrated by roentgenographic... |
| Purpose : Infants undergo critical and rapid stages of growth. Optimal nutrition during this period is therefore essential. Iron deficiency is especially impotant and common. The aim of this study was to determine the present status and problems of milk feeding methods in infants with iron deficiency anemia and, futhermore, to develop appropriate feeding guidelines for adequate iron nutrition. Latate is the end product... |
| We experienced two cases of factor XI deficiency in a 5 yearr 7 month-old girl, whose chief complaint was coke-colored gross hematuria, and her 9-year-old elder sister. The laboratory findings were prolonged aPTT and prominent deficiency of factor XI. The gross hematuria of the index case was identified due to acute poststreptococcal glomerulonephritis. So we report two cases of factor... |
| Purpose : To assess whether pretreatment IGF-I SDS and pretreatmental bone age are useful indicators in the response of rhGH therapy in children with GH deficiency(GHD), the relationship between pretreatment IGF-I SDS, delay in bone age and the growth response during the first year of rhGH treatment was analyzed. Methods : This study included 12 children with GHD. We measured IGF-I levels by RIA... |
| Purpose : Medium chain acyl-CoA dehydrogenase(MCAD) deficiency is an autosomal recessive disoder of β oxidation of fatty acids and characterized by episodic hypoglycemia, vomiting, convulsion, encephalopathy, apnea, and sudden death related to fasting or infection resembling Reye syndrome or sudden infant death syndrome. In acute stage, mortality rate is very high and survivors have significant risk of developmental disability and chronic somatic illness. However,... |
| Purpose : The aim of this study was to evaluate the long-term growth response to growth hormone therapy in 18 children with naive growth hormone deficiency. Methods : The subjects of this study were aged 3-13.5 years and each patient continuously received GH(EutropinⓇ) 0.45-0.7IU/kg/week, intramuscularly or subcutaneously in 3-7 divided dose for up to 7 years. Standard auxologic assessment was carried... |
| Lymphoid interstitial pneumonia(LIP) is an uncommon process in childhood, and is characterized by interstitial accumulation of mature lymphocytes, plasma cells and reticuloendothelial cells. LIP is believed to have autoimmune or immunologic pathogenesis, because it is frequently associated with acquired immune deficiency syndrome, Epstein-Barr virus infection, common variable immunodeficiency. The patient described in this report had idiopathic thrombocytopenic purpura, Evans syndrome... |
| Hereditary stomatocytosis is a rare congenital hemolytic anemia, named after mouth shaped or stomatocytic erythrocyte morphology. In this report, we present a case of a hereditary stomatocytosis in a 1 month old boy. During the initial identification process, we overlooked the morphology of the RBC in peripheral blood smear and tentatively diagnosed it to be a hereditary spherocytosis case. In order to... |
| Nezelof's syndrome(combined immunodeficiency with immunoglobulin) is a hereditary primary immunodeficiency characterized by recurrent chronic pulmonary infections, oral and cutaneous candidiasis, failure to thrive, chronic diarrhea, skin infection, urinary tract infection, gram-negative sepsis, severe progressive varicella infection, lymphopenia, diminished lymphoid tissue, abnormal structure of the thymus, and presence of normal or increased levels of one or more of the major immunoglobulin classes, but with impaired antibody... |
| Purpose : For diagnosis of growth hormone(GH) deficiency in short stature, peak growth hormone levels after pharmacologic stimulation are usually used. In this study, we measured serum IGFBP-3, which is a major binding protein in serum and is considered to be GH-IGF-I axis dependent, levels by radioimmuno assay(RIA) in sera from normal short stature(NSS) children, and patients with GH deficiency children to clarify the... |
| Helicobacter pylori has become apparent that this organism is an etiologic agent of gastrits and peptic ulcer disease in humans. We present four cases of iron deficiency anemia, not specific causes, in 3 males and 1 female patients with Helicobacter pylori infection, who were treated with antibiotics and iron supplemented therapy. Diagnosis was confirmed by endoscopic biopsies and hematologic findings. A brief review of related... |
| Hemorrhagic disease of newborn by combined blood clotting factor deficiencies is very rare. Combined deficiency of factor V and factor VIII is the most common form among these cases, and inherited by autosomal recessive trait. Clinical findings are easy bruising, posttraumatic bleeding and bleeding after tooth extractions, and the main laboratory findings are prolonged partial thromboplastin time (PTT) and prothrombin time (PT) that are... |
| Purpose : The insulin like growth factors (IGFs) circulate complexed to IGF-binding proteins(IGFBPs). IGFBP-3 is the major circulating IGFBP and is found primarily as a 150 kDa complex which contains an acid labile subunit(ALS), IGFBP-3, and IGF-I or IGF-II and is considered to be growth hormone(GH) dependent. In this study, we measured serum IGF-I, IGFBP-3 and 150 kDa levels in sera of growth hormone... |
| A 12-year-old girl with hypertension, hypokalemia, cystic ovaries and absence of secondary sexual development is presented. Hormonal study revealed very low levels of cortisol, testosterone, estrogen, and high levels of progesterone, deoxycorticosterone, corticosterone, FSH and ACTH. Following treatment with dexamethasone and estrogen, the levels of the latter group remarkably decreased. Serum potassium level and blood pressure also became normal. With... |
| Purpose : Although the chronic sinusitis is one of the most common and troublesome respiratory diseases in children, the pathogenesis still remains unclear. It is suggested that many of the immunologic factors including allergic conditions may contribute the nasal inflammatory changes. This study was designed to evaluate and demonstrate the possible role of various immunologic factors on the pathogenesis of... |
| Purpose : Iron deficiency anemia(IDA) is the most common nutritional deficiency. in young infants. This study was designed to determine a correlation between the duration of breast feeding and the severity of IDA, and to determine the recommending start point of iron fortified diet. Methods : The correlation between duration of breast feeding, and severity of iron deficiency. and IDA was... |
| Purpose : At present, to determine growth hormone(GH) deficiency in short stature, many provocative tests using various pharmacologic agents such as clonodine, L-dopa, insulin should be done. These are not only complicated, but also misleading in some patients. And so, in search of a simple method of detecting GH deficiency that may replace the complicated stimulation test, we measured basal... |
| Purpose : To study the incidence of iron deficiency anemia we conducted blood tests with questionnaire regrarding weaning status. We tried to figure out the relation between iron deficiency anemia and financial and educational levels of their parents. The purpose of this study is to emphasize the importance of screening for iron deficiency anemia at well baby clinic and educate mothers... |
| Purpose : To investigate the diagnostic significance of urinary growth hormone measurement and compare with the urinary GH values in different methods of urine collection, this study was performed in patients with short stature. Methods : Measurement of urinary GH excretion was performed in 9 children with complete growth hormone deficiency (GCD), in 19 children with partial growth hormone deficiency (GPD)... |
| We have experienced 4 and 5-week-old male neonates presented low T4 and normal TSH l-evel by filter paper mothod for neonatal screening, finally diagnosed typical TDG deficiency wit h TBG radioimmunoassay, paper electrophoresis, and autoradiography. Two neonates have ha d no specific complaints and lived well until now. Congenital TBG deficiency is an X chromosome linked inhcritant disorder characterized by l... |
| Purpose : This study was performed kto evaluate the High frequency flow interruption(HFF I) can prevent the lung injury and achieve a bettre oxygenation than conventional ventilation(C V) in surfactant deficient lungs when associated with lung volume recruitment and maintenance, and the need of background intermittent mandatory ventilation(IMV) to prevent the progressive atelectasis during HFFI. Methods : Surfactant deficiency was produced... |
| Tetrahydrobiopterin(BH4) deficiency is a rare type of hyperphenylalaninemia and usually leads to a progressive neurologic deterioration despite early dietary control of blood phenylalanine concentration. We experienced two cases of BH4 deficiency in brother and sister, confirmed by biochemical study of blood and urine. They had suffered from a progressive neurologic illness such as mental retardation, severe hypotonia, seizure, and athetotic movements... |
| Purpose : This study was conducted to get baseline data to establish a guideline for the infant nutrition by observing the feeding practice and nutritional intake during infancy and evaluationg the nutritional status of iron and lipids at 12-month-old infants. Methods : This survery was cnducted from July to November, 1992. Subjects were healthy infants who were brought to Yeungnam University... |
| Eosinophilic gastroenteritis(EG) is a rare disease characterised histologically by eosinophilic infiltration of the gut wall. The clinical features depend on which layer and location are involved. Patients may be divided into three clinical groups as predominantly mucosal, muscle layer, or subserosal disease based on the histological site of eosinophilic infiltration of the bowel wall, although there is lften considerable overlap.... |
| To elucidate the complex of insulin-like growth factor binding proteins (IGF-BPs) in short stature patients, we carried out a prospective study on three patients who were diagnosed as complete GH deficiency at the department of pediatrics form July 1992 to June 1993. The results were summarized as follows: 1) Two circulating IGFs complexed to specific binding protein existed in normal serum. Binding... |
| Congenital adrenal hperplasia is inherited disorder of adrenal steroidogenesis. 21-hydroxylase deficiency is the most commone enzymatic defect and is divided into classic and late-onset or nonclassic forms. Both classic and non-classic 21-hydroxylase deficiencies are inherited in a recessive manner as allelic variants. But it is rare that happened in twin infants. Chief complaints of affected twins in our case were ambiguous... |
| To validate the diagnostic significance of red cell indices in non-anemic iron deficiency and iron deficiency anemia, complete blood count, serum iron, total iron binding capacity, and serum ferritin were measured in 208 middle school girls between 13 and 15 years age. We used Reciever Operating Characteristic (ROC) curve to compare the diagnostic significances of various red cell indices (MCV,... |
| To determine the impact of intake large amount whole cow's milk (WCM) on iron status during early childhood(18~36 months), selected indices of complete blood count (CBC) and iron status were compared between the WCM large amount intake group (n=20) and small amount intake group (n=20). WCM large amount intake children's mean hemoglobin and mean hematocrit were 9.6¡¾1.7g/dl and 30.8¡¾4.5% respectively. These... |
| Urinary growth hormone (GH) excretion was quantitated in 12-hr overniaght urine collections obtained from 13 children with complete growth hormone deficiency(CGHD), 6 children with partial growth hormone deficiency(PGHD), 5 children with short stature and normal GH provocation tests(NSC), and 5 normal control children (NC) to investigate whether the measurement of urinary GH can clearly separate the PGHD and CGHD groups... |
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