Angioimmunoblastic lymphadenopathy is a lymphoma like disorder consisting of generalized lymphadenopathy, hepatosplenomegaly, skin rashes, often associated with dysgammag- lobulinemia. We have experienced recently 3 year old female complaining of generalized lymphadenopthy, fever, hepatomegaly and hypergammaglobulinemia, and was proved as angioimmunoblastic lymphadenopathy histologically. Patient was treated with combined chemotherapy with COMP and responded well clinically in 4 months. The brief review of the literature was made. |
Islet cell adenoma is a rare cause of severe neonatal hypoglycemia. A newborn infant with severe symptomatic hypoglycemia had persistent hypoglycemia in spite of therapy with parenteral glucose solution. Islet-cell adenoma of mixed islet-acinar type was found when subtotal pancreatectomy was performed at 42 days of age. Following surgery she had a transient hyperglycemia and required isulin for 40 days. She has remained normoglycemic but... |
The clinical study on 28 cases of ABO HDN who were diagnosed at Pediatric Department of Severance hospital and Yong Dong Severance hospital, Yonsei University for last 5 months was. And in these cases, heat elution test in combined with direct Coombs test using standard techniques were performed to evaluate the clinical significance of serological diagnosis. The following results were obtained. 1) Of 28 ABO HDN... |
Recently we have experienced a case of infantile cortical hyperostosis. The patients was 2 months old male who complained irritability, soft tissue swelling of both lower extremities. X-ray showed periosteal new bone formation on both tibiae. Bone scan revealed hot uptake at the lesion. We are reporting this infantile cortical hyperostosis with reviewing literatures. |
Histiocytic medullary reticulosis (HMR) is a clinicopathologic syndrome characterized by its acute onset and relentless progression to death within a few months. Major clinical features are fever, wasting, generalized lymphadenopathy and hepatosplenomegaly. In the terminal stages, Jaundice, purpura, anemia and leukopenia are common. Cardinal pathologic features are diffuse proliferation of atypical, neoplastic, erythrophagocytic histiocytes and their precursors throughout the reticuloen- dothelial system. Here, we present 2... |
This study was undertaken to eluidate lipoprotein patterns in patients with childhood nephrotic syndrome by response to initial steroid therapy, serum albumin level in MCNS and other histologic classifications. The result were summerized as follows. 1) There were 19 cases (52.8%) of abnormal lipoprotein patterns by Fredrickson classifications, type Ila were 6 cases (16.7%), type lib were 1 cases (2.8%) and type IV were 12 cases... |
ABO hemolytic disease in newborn due to ABO incompatibility between mother and infant is one of common cause of neonatal hyperbilirubinemia and early detection has been emphasized due to its possible neurologic sequelae. Many studies reported prevalence difference of ABO hemolytic disease in various races, which could be ascribed to different frequency of ABO genes in races. From Feb. 1986 to May 1987 full... |
Acute megakaryoblastic leukemia is an uncommonly recognized disorder that is characterized by rapidly progressive proliferation of atypical megakaryocytes and their precursor cells, and fatal course. Abnormalities in chromosome 21 may have a more than random relationship to acute megakaryoblastic leukemia. The authors reports a case of acute megakaryoblastic leukemia in a 2 month old female patient who was admitted for evaluation of hepatosplenomgaly. Multiple organ infiltration... |
A 2-day-old male infant was admitted to the Incheon Christian Hospital due to severe jaundice. He was a third baby and his Rh phenotype was CcDEe. The direct antiglobulin test of the infant was strong positive. Eluates that were made from the infanfs elder brother and sister were both CDe. Her two previous infants showed no evidence of erythroblastosis. She had a past... |
To evaluate the influence of nephrotic syndrome on the thyroid function test in children, serum T3, T4, free T4, TSH, TBG and rT3 were measured using radioimmunoassay in 14 clinically euthyroid children with nephrotic syndrome who were admitted to Department of Pediatrics, Pusan National University Hospital from July, 1986 to August, 1987. Control group was 15 children who visit our outpatient department for minor... |
We studied 197 cases of patients with goiter who visited Department of Pediatrics, Yonsei University College of Medicine from January 1980 to June 1987. For each patient, serum T4, free T4, T3, TSH, antithyroid antibody, and antimicrosomal antibody were analyzed, thyroid scan was performed, and radioiodine uptake was measured. Following results were obtained: 1) Male to female ratio of 197 cases of goiter was 1:7.5, predominent... |
PRA’s in peripheral vein, both renal veins, and inferior vena cava (IVC) were measured in 25 normotensive children without renal disease undergoing cardiac catheterization for their underlying cyanotic congenital heart disease. And we presented the results as reference values/ranges of PRA in children. 1) There was no significant difference in PRA between both side renal veins at any absolute PRA levels with a mean ratio (RT/LT)... |
Only IgG anti-A, or Anti-B cross the placenta and are responsible for the occurrence of ABO hemolytic disease of newbom(HDN). We have conducted the role of IgG anti-A or anti-B in mothers to occurrence and severity of the disease with were related to the level of antibodies in pathogenesis of ABO-HDN. This report presents the relation between the degree of bilirubin, hemoglobin concentration in ABO-HDN... |
Chromosomal studies were carried out in 730 patients at the Dept, of Pediatrics and Genetics, Hanyang University Hospital, form December 1979 to April 1986. A statistical assessment was carried out in 244 cases who have chromosomal aberrations. The following results were obtained: 1) Chromosomal aberrations were observed in 244 cases among 730 cases, so the propotion of chromosomal aberrations was 33%. 2) Age distribution revealed 53% of chromosomal... |
We studied prospectively whether events that result in meconium staining may be associated with additional abnormalities that are not clinically apparent associated by measurements of urinary concentrations of β₂-microglobulin indicating renal proximal tubular functions. Among 61 fullterm, healthy neonates delivered at Hanyang University Hospital during the period of September 1986 through April 1987, urinary concentration of β₂-microglobulin and creatinine were measured during the immediate perinatal... |
Cleidocranial dysostosis is a rare, inherited abnormality which affects the development of mem- bransous and cartilaginous bone. Authors report a case of cleidocranial dysostosis with leukemia in a 10 year old boy. The review of the literature was made briefly. |
We report 7 cases of Group B streptococcal meningitis and sepsis. Six of the patients were admitted to the Department of Pediatrics, Severance Hospital and Youngdong Severance Hospital in 1986 and one was admitted in 1979. The age of onset ranged from 10 to 34 days of age and all were cases of late onset. The Clinical findings were fever, seizure, poor feeding,... |
Aortic valve stenosis was relieved by balloon dilatation during cardiac catheterization on 9 children aged 1 month to 15 years. Aortic valve diameter was estimated by two dimensional echocardiography to assist in the choice of balloon size. Before dilatation the transaortic systolic pressure gradients ranged from 60 mmHg to 177 mmHg, (mean: 91 ±38 mmHg). There were signifeant reduction in the transaortic systolic pressure... |
Hypoplastic left heart syndrome is a rare, and fatal congenital heart disease. Most of the patients with this syndrome die within the 1st month of life. Recently we experienced a hypoplastic left heart syndrome in a child who was unusually living without surgical intervention for 3 and half years. The unique clinical course might result from a number of favorable hemodynamic factors and intact... |
We experienced a case of truncus arteriousus associated with interrupted aortic arch, ventricular septal defect and patent ductus arteriosus. A 5 month old boy had complained of rapid respiration and feeding difficulty since birth. There was no visible cyanosis or clubbing toes and nails. There was a grade 2 〜3 ejection systolic murmur with maximum intensity at the left lower sternal border. Diagnosis was... |
Statistical analysis of pediatric diseases in patients admitted to the department of pediatrics in 34 hospitals having pediatric residency program for 2 years from Jan. 1985 to Dec. 1986 was underwent for the purpose of assessing the disease pattern in the pediatric aged people in Korea. The results obtained were as follows 1) Total number of inpatients was 112,309, of which 69,266 were male and... |
Congenital nephrotic syndrome is an uncommon disorder, which is caused by a heterogenous group of renal diseases with variable etiology and natural history. It may be inherited, sporadic, acquird or a part of a general malformation syndrome. This disorder has no single or pathological feature for pathognomonic finding, and there is no ultimate curative treatment except renal transplantation. We experienced a case of congenital... |
Thrombotic thrombocytopenic purpura is clinically characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, renal disorder and transient bizarre neurological symptoms. Its essential histological lesion consists of numerous complete or, more often, incomplete occlusions of the arterioles and capillaries by hyaline material. We have experienced a case of thrombotic thrombocytopenic purpura in infancy. The patient was a one year and 4 month-old who complains petechiae on the... |
A clinical assessment of the 30 patients with extrahepatic biliary atresia who were admitted to the Department of Pediatrics and pediatric Surgery, Yonsei University College of Medicine from January, 1979 to January, 1987 was performed. In 21 patients who underwent hepatic portoenterostomy, the relationship between outcome and surgery was analyzed. Finally, collelations between lonterm prognosis and the histopathology of biopsied liver tissue, postoperative bile... |
Acute hepatic porphyria may be an inborn error of metabolism characterized biochemically by the excessive hapatic over-production and urinary excretion of porphyrin precursors and clinically by episode of acute neuro-visceral dysfunction. Acute porphyria is very rare in childhood. We experienced a case of acute hepatic porphyria in childhood age, confirmed by characterized clinical features of neuro-visceral dysfunction and biochemical findings in blood and urine... |
To assess the clinical significance of histopathologic variants of minimal change nephrotic syn- drome (MCNS) by light microscopic findings, 148 biopsy proven MCNS patients, who were admitted to Seoul National University Hospital between the period from January 1976 to December 1986, were analyzed according to clinical findings, steroid responsiveness, and follow up status in relation to the pathologic variants. The followings are summary and conclusion. 1) Significantly... |
The authors experienced a case of benign cystic sacrococcygeal teratoma in a 2 month-old female infant. The cystic mass was located at presacral area without external presentation, causing left renal hydronephrosis and ureterectasis. Surgical excision of the mass was performed successfully. The literature regarding the subject was reviewed. |
We experienced a case of congenital subglottic stenosis in a male baby of 2 month old. He surffered from respiratory difficulties since 40 days after birth. Initially, he had been considered as a stridor because of the symptom of upper airway obstruction did not improved for 4 months, so we started to assess the lesion of subglottic area since 15 days after admission,... |
Hereditary elliptocytosis is characterized by elliptically shaped erythrocytes in peripheral blood and known to be transmitted as an autosomal dominant trait, but in some cases, inherited recessively. The clinical and hematologic expressions are variable range from healthy person with normal red cell morphology to severe hemolytic anemia. Recently, the authors experienced all three offsprings shown non-hemolytic hereditary el- liptocytosis, whose parents were healthy persons with... |
A 4 day old male patient was admitted to our hospital due to jaundice, poor feeding and abdominal distention. His clinical features and peripheral blood smear revealed congenital myeloblastic leuke- mia which confirmed by autopsy. Chromosome study showed 47, XY, +21 in Karyotype. We report this case with review of literatures. |