| A synophthalmia, another form of cyclopia, in which the element of the two eyes are partially fused to form an apparently single eye in the middle of the forehead. The synophthalmia is a result of complex, neural plate misdevelopment syndrome involving the eye, brain, skull and face. It is well known that synophthalmia is due to heterogenous causes, most of... |
| Congenital hypoprothrombinemia is a rare congenital coagulation defect. The clinical signs are manifestation of generalized bleeding tendency such as; mucosal bleeding, hypermenorrhea and post tooth extraction hemorrage. It is associated with prolongation of PT and PTT with normal thrombin time and decreased serum prothrombin level. A case with congenital hypoprothrombinemia was experienced by the authors. A 36 days old male baby... |
| The eosinophil is the major cell responsible for the inflammatory reaction in bronchial asthma. Secretion of granular components is probably of considerable importance for the inflammatory effects of the eosinophils, Eosinophil cationic protein (ECP) is the one of the secretory components of the eosinophil granule and is considered as an activation marker of eosinophil, Blood eosinophil count, serum concentration of... |
| Fifty five children with encoscopically proven esophageal varices form Jul. 1987 to Dec. 1992 were analyzed for endoscopic and clinical characteristics. The results were as follows : 1) According to etiological classification of portal hypertension in 55 cases, 35 cases showed intrahepatic causes (64%) and cases had extrahepatic causes (36%). The most common clinical manifestation on admission was hematemesis (42%) and abdominal mass... |
| 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 symtons, such as diarrhea, irritability,... |
| We examined the effect of various levels of dietary protein on long term prognosis of Adriamycinephropathy of S-D rat, fed with high protein(30%), intermediately low(10%), and strictly low(5%) protein diet for 15 weeks 1) In rats fed with strictly low protein diets(5%), proteinuria and serum creatinine decreased and creatinine clearance and histological changes were relatively well preserved. But hypoproteinmia and weight... |
| Eosinophils play a major role in the airway inflammation in asthma. To werify an association between airway responsiveness of asthmatics and eosinophil and/or ECP, we measured serum ECP in 23 children with atopic asthma during acute attacks and stable, and in 10 normal healthy controls. Peak flows were monitored in 15 children. The data suggest that serum ECP levels were higher... |
| Together, neonatal hepatitis and biliary atresia make up approximately 70 to 80% of the causes of neonatal cholestasis. Biliary atresia must be differentiated from neonatal hepatitis as soon as possible in order to institute early surgical intervention. We performed this study to examine whether the measurement of the serum α-fetoprotein(AFP) and thyroxine-binding globulin(TBG) was useful for differentiating these two conditions.... |
| The late T cell activation gene, 519, is expressed in antigen specific, growth factor dependent T cell lines and clones but not in T or B cell tumors, other hematopoietic cells, tonsil, muscle, lung, or liver. Resting peripheral blood ymphocytes(PBLs)express little or no 519mRNA, but levels increase dramatically 5~7 days after activation with alloantigen or mitoen. Four alternatively spliced transcripts... |
| A study was made on 50 cases of epileptic children who had been observed at the pediatric department of Chungnam National University Hospital and Taejeon Eul Ji Hospital during the period from July, 1986 to June, 1991 The result are as follows : 1) Epilepsy was most frequent in children from 1 yar to 10 year of age(80%). Epilepsy was commoner in... |
| Intestinal lymphangiectasia is a primary or secondary disorder of the gastrointestinal tract, which is associated with lymphatic dysfuction and protein-losing enteropathy. It's clinical manifestations vary widely, but the main symptoms are abdominal distention, edema, abdominal pain and growth failure. We described a 15-yr-old boy, who suffered from above symptoms since third year of his age and diagnosed by duodenal endoscopy which... |
| This study reviewed 66 episodes of septicemia occurring in 56 children with neoplastic disease at Seoul National University Children뭩 hospital over 31 month period. The most frequent organisms causing 45 episodes in neutropenic patient were Escherichia coli (28%), Klebsiella species (18%), Pseudomonas species (9%), polymicrobic (9%,), and Staphylococcus aureus (7%). The overal mortality was 13% (5/39). The most frequent organisms... |
| Fukuyama type congenital muscular dystrophy is characterized by mental retardation, progressive muscle weakness and hypotonia, joint contracture and seizures in half of the reported cases. The usual CT abnormalities are dilated cerebral ventricles and Sylvian fissures and lucency of cortical white matter. The most consistent pathologic findings in the brain are polymicrogyria and pachygyria, caused by a disturbance of migration... |
| Infection by Listeria monocytogenes, an uncommon pathogen in immunocompromised host, has been reported in neonates, pregnant women and adults with defective cell mediated immunity but not in children in Korea. It can cause sepsis, meningitis, abortion, premature labor, intrauterine fetal infection. etc. We have experienced L. monocytogenes sepsis and/or meningitis in three children under immunosuppresive therapy for lymphoma-leukemia, cytophagic histiocytic... |
| The endodermal sinus tumors are rare and highly malignant germ cell origin tumors occuring in both gonadal and extragonadal tissue. We have experienced a case of endodermal sinus tumor mixed with benign teratoma occuring in the common bile duct. It is a very rare site for endodermal sinus tumor. Serum α-fetoprotein was well correlated with clinical course. |
| Hutchinson-Gilford Progeria Syndrome is an extremely rare condition that was initially reported by Jonathan Hutchinson in 1886 and further described by Hastings Gilford in 1904. Clinical manifestations are evident by the first or second year of life with the physical characteris-tics of the elderly. Progeric patients ordinarily develop atherosclerosis and die of cardiac or cerebral vascular disease between 7 and 27... |
| 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... |
| The authors observed the clinical findings of 36 patients with meconium aspiration syndrome(MAS) to evaluate the effect of early appropriated meconium suctioning on the morbidity and mortality of the syndrome. Patients in the study group, 20 babies born in Chonnam university Hospital, received appropriate meconium suction through endotracheal tube during and rig-ht after the delivery, while the patients in the... |
| A case of Holoprosencephaly, alobar type , was presented here and 13-month follow-up has been made. He had dyspnea, polikilothermia, frequent atypical seizures, hypernatremia & pitressinresponsive Diabetes insipidus as clinical manifeststions. Chromosome studies showed normal male karyotype (46XY). The diagnosis was confirmed by brain CT scan. A brief review of the related literature was made. |
| Prostaglandin E1 (PGE1) was administered to 12 neonates with cyanotic congenital heart disease in whom pulmonary or systemic blood flow were entirely or significantly dependent on persistent patency of the ductus arteriosus. 10 neonates responded favorably to PGE1, by showing significant increase of PaO2 after PGE1 infusion but remaining 2 neonates with hypoplastic left heart syndrome failed to respond. The... |
| Transient myeloproliferative disorder has been reported in infants with Down’s syndrome. This disorder is clinically and hematologically indistinguishable from congenital leukemia. In contrast to congenital leukemia, complete clinical and hematological recovery occurs within weeks to months of diagnosis without antileukemic treatment. We experienced a case of Transient myeloproliferative disorder with Down’s syndrome. This 4-day-old female neonate showed jaundice, hepatomegaly, fever, dyspnea, mongoloid face and congenital heart... |
| Holoprosencephaly is a congenital malformation complex involving developmental failure in normal cleavage of the forebrain into hemispheres and varying degrees of median facial deformities. We experienced a case of lobar holoprosencephaly in a one day old girl who showed median facial deformities and reviewed the references concerning holoprosencephaly briefly. |
| This study was conducted to investigate the effects of CNS prophylaxis with 1800 cGy cranial irradiation and intrathecal methotrexate (MTX) chemotherapy on intellectual function of the chil- dren with acute lymphocytic leukemia (ALL). Thirteen longterm survivors with ALL who were treated with 1800 cGy cranial irradiation and intrathecal MTX for CNS prophylaxis at pediatric department of Yeungnam University Hospital from May, 1983 to November, 1988... |
| There is continuing interest in the isotype response of human serum antibodies reactive with dietary proteins. This study was performed to clarify the humoral immune responses against cow’s milk proteins, 分-lactoglobulin and a-lactalbumin. Milk protein specific IgG, IgA, IgM, and IgG subclasses were measured using ELISA in the blood of various groups of age. The results were as follows: 1) Milk protein specific immunoglobulins were higher... |
| Almost of all patients with neurofibromatosis type I show caf6-au-lait macules on their skin. These macules are thought to be early sign of neurofibromatosis in childhood. We experienced a case of neurofibromatosis type I in a 10-year-old-boy who did not have caf6-au-lait macules, but progressive psychomotor retardation, short attention span, hyperactivity and megalencephaly. The brain C-T findings showed diffuse cerebromalacia and ventriculomegaly. We confirmed the... |
| This is brief report of a case of prune belly syndrome associated with holoprosencephaly in one-day old neonate. At birth, physical examination revealed skull bone defect, anophthalmia, severe hypotelorism, arhinia with proboscis, micrognathia, webbed neck, severely dilated abdomen with thin wall, agenesis of external urogenital organs and anus, equinovalgus and genu valgus. Autopsy findings were alobar holoprosencephaly with hydrocephalus, hypoplasia of lung and ribs, markedly dilated... |
| This study was undertaken to evaluate the prognostic factors of recurrence and renal involvement in Henoch-Sch公nlein purpura. Total 110 cases of anaphylactoid purpura below 15 years of age who admitted to Pediatric Departmen of Kyung Hee University for 8 years from January 1st 1979 to December 31st 1986 were analyzed clinically and statistically. The results were obtained as follows. 1) The peak incidence of age was 4... |
| The present study was performed to define the prevalene of asymptomatic hematuria, proteinuria,and glucosuria. A total of 5011 primary school children in Chonju city were examined with test strip urinalysis during the period of one month from June to July 1989. Test strip (Hema-Combistix) was used in this examination. The results were as. follows: 1) Prevalence of hematuria, proteinuria and glucosuria were 0.8% (40 children), 0.78%... |
| Children with Down syndrome have an increased incidence of acute leukemia. Infants with Down syndrome are also at risk of developing a transient myeloproliferative disorder indistinguishable from acute nonlymphocytic leukemia (ANLL) except by its eventual clinical recovery. We observed 11 patients with acute leukemia or transient myeloproliferative disorder in Down syndrome who had admitted to the Departmetn of Pediatrics, Seoul National University Children’s Hospital,... |
| The omphalomesenteric duct in the embryo is a communication between the yolk sac and primitive gut. The duct closes by six to eight weeks of intrauterine life and disappears soon after. Patent omphalomesenteric duct is a congenital anomaly which occurs when there is incomplete closure of the duct during this period. The authors experienced a cases whereby a 29-day old male baby had patent... |
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