Focal segmental glomerulosclerosis is the renal histopathologic lesion observed in 10% of children with idopathic nephrotic syndrome. Complications include infection, thrombosis, hypocalcemia and adverse effect of steroid use. The incidnce of thrombosis reported ranges from 10~40%. The pathogenesis are changes in coagulation system, decrease of Antithrombin III, increase platelet aggregability and steroid or diuretics use. These changes take place passively... |
T cell lymphoblastic lymphoma is characterized by immature lymphoid cells that are indistinguishable from the lymphoblasts and prolymphocytes of acute lymphoblastic leukemia. Several characteristic clinical features of lymphoblastic lymphoma, with include a high male-to-female ratio, a relatively high incidence in older children and young adults, the frequent presence of mediastinal involvement at the time of diagnosis. Also, this disease is... |
Isolated angiitis of the central nervous system is a rare clinicopathologic entity characterized by vasculitis restricted to the vessels of the central nervous system without other apparent systemic vasculitis. It manifests headache, higher cortical dysfunction, focal neurologic dysfunction and cranial nerve palsies. We experienced a case of isolated angiitis of the central nervous system in 6 year-old girl who was admitted... |
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare disease characterized by fever, hepatosplenomegaly, cytopenia and non-malignant lymphohistiocytic infiltration with hemophagocytosis in reticuloendothelial organs. We experienced three cases of FHL in identical male twins and their younger brother who presented with fever and severe hepatosplenomegaly. Cytompenia, elevated serum transaminase and low serum albumin levels, hypertriglyceridemia were common laboratory findings of them. One of... |
The etiology of nephrotic syndrome is unknown. The characterization were proteinuria, hypoalbuminemia, generalized edema and hyperlipidemia. To assess the recurrence factors in the nephrotic syncrome, we measured serum immunoglobulin(IgG, IgA, IgM), albumin, complement, cholesterol and the 24-hour total urine protein at the initial relapse of nephrotic syndrome. Each data of frequent and infrequent relapsed nephrotic syndrome were compared. In total... |
The aim of this study was to determine the applicability of reverse transcription and polymerase chain reaction(RT/PCR) for routine diagnostic use and for the detection of enteroviral mentigitis, Primers were selected in the highly conserved part of the 5'-non-coding region of the enteroviral genome. Enteroviral RNA was detected by the RT/PCR in routinely collected cerebrospinal fluids(CSF) of patients with aseptic... |
Maffucci's syndrome is a very rare, congenital and non-hereditary mesodermal dysplasia manifested by multiple enchondromas and soft tissue hemangiomas. Since Maffucci had reported this syndrome in 1881, there have been more than 100 cases reported, and also there has been reported that Maffuddi's syndrome has various interstitial tumor. However there hasn't been any report about Maffucci's syndrome with testicular teratoma.... |
Purpose : We evaluated the protective effect of dexamethasone (DX) administration on brain damage produced in a perinatal model of cerebral hypoxia-ischemia in the rat. Since hyperglycemia has been shown to reduce hypoxic-ischemic brain injury (HI) in immature rat, we investigated the role of glucocorticoid-induced hyperglycemia in the neuroprotective mechanism of DX. Methods : Hypoxic-ischemic brain injury in 7-day-old rats was... |
A12-year-old boy developed cholestatic hepatitis with Steven-Johnson syndrome following the use of amoxicillin. The skin lesion and general condition were improved over 2 weeks, but jaundice was gradually aggrevated. We performed liver biopsy, on 30th hospital day, which showed cholestatic hepatitis. The patient improved gradually and liver function was normalized 5 months later. |
Pulmonary blastoma is a rare type of malignant lung tumor comprised of epithelial and mesenchymal elements reminiscent of fetal lung. Mesenchymal element may show various patterns of differentiation toward mature tissue, such as cartilage, smooth muscle, and bone. Rhabdomyoblastic differentiation in pulmonary blastoma is quite rare. Our case of pulmonary blastoma with rhabdomyoblastic differentiation documented by electron microscopy and immunohistochemistry. Usually... |
After aseptic or septic meningitis, some neurologic complications such as convulsions, delirium, rigidity, cerebral infarctions and cerebral hemorrhage can be developed. The cerebral infarction after meningitis is caused by arterial or venous occlusions. Involvement of small perforating arteries leads to ganglionic infarcts while severe sapsm of major vessels may lead to massive infarctions in the distribution of middle and/or anterior... |
Various anatomical defects have been described in the surviving twin who had a stillborn, macerated monozygotic co-twin Disseminated Intravascular Coargulation. The etiology is thought to be placental transfer of emboli or thromboplastic material through placental vascular anastomoses. We experienced a case of monozygotic twin with deceased co-twin at 30 weeks of gestation and confirmed to have antenatal periventricular germinal matrix and... |
Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have been associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result... |
A retrospective study was conducted to investigate the effects of CNS prophylaxis with 1,800cGy cranial irradiation on standing height growth of children with acute lymphoblastic leukemia(ALL). Seventeen longterm survivors (DFS over 5 years) of ALL and Non-Hodgkins Lymphoma who had been admitted at pediatric department of Inje University Seoul Paik Hospital from January 1982 to September 1988 were studied. The long term... |
Pulmonary vein velocities have recently been estimated in conjunction with mitral flow velocities to increase our understanding of ventricular filling. The advent of transesophageal echocardiography with pulsed doppler imaging capability has provided a method by which both the mitral valve and pulmonary vein velocities can be easily recorded because of the posterior approach providing unimpeded interrogation of cardiac structures. The purpose... |
Primary cutaneous aspergillosis is rare. In this report we describe primary skin infection by Aspergillus in 9 children with leukemia. The skin lesion was characterized clinically by erythematous macule and papule associated with pain and itching, followed by a rapid progression to ulcer and central black eschars with raised erythematous border at the site of venipuncture, insertion of intravenous cannula,... |
To study the site of release and factors affecting the releases of the atrial natriuretic peptide (ANP) in the pediatric patients, we measured the plasma ANP concentrations in 23 normal healthy children, 19 patients with congestive heart failure and 16 patients with cardiac disease undergoing diagnostic cardiac catheterization. The following results were obtained. 1) The plasma ANP concentrations of the vein (226.3¡¾74.9... |
The congenital diverticulum of ventricle is a rare cardiac malformation arising from the left or right ventricle, the former being more common. Ventricular diverticulum is usually associated with other anomalies including intracardiac, midline thoracic, diaphragmatic and abdominal wall defect. The authors experienced a case of congenital diverticulum of left ventricle in nine month-old female infant. Left Blolock-Taussig shunt operation was done... |
We reviewed retrospectively the medical records of 229 cases of tuberculosis, who had been diagnosed clinically and admitted to the department of pediatrics, Seoul National University Children's Hospital, during the period of 6 years from October, 1985, to October, 1991. Patients with tuberculosis occupied 1.2%. of the hospitalized patients. Forty-three percent of the patients were under 3 years of age. Principal... |
We analysed retrospectively pharmacokinetic parameters of gentamicin and amikacin in 44 and 58 Korean pediatric patients, respectively, with normal renal function. Pharmacokinetic parameters were calculated from two concentrations in serum by method of Sawchuck. There was wide individual variation in peak serum concentrations of gentamicin and amikacin. Administration of the usually recommended doses yielded subtherapeutic concentration in 47% and 82%,... |
The Kidney size is of special interest during the diagnostic work up of acute lymphocytic leukemia. But is still uncertain whether a finding of kidney enlargement at presentation has long-term prognostic value. We therefore reviewed the kidney size in children with acute lymphocytic leukemia by abdominal ultrasonograms at the time of diagnosis (n=54) and examined if there was any statistical... |
Congenital dyserythropoietic anemia Type II (herditary erythroblatic multinuclearity with positive acidfied serum test;HEMPAS) is characterized by binuclearity, multinuclearity, pluripolar mitoses, karyorrhexis of normoblasts, and the presence of abnormla antigens on the red cells. We experienced a case of HEMPAS in a 2 month old girl patient who had an intermittent fever, abdominal distention with palpable liver & spleen, and generalized jaundice. The... |
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,... |
Sacrococcygeal teratoma is a rare tumor, it occurs once in every 40,000 live births. Most of the reported cases appear in female infant (M:F=1:4). They deserve clinical attention because they are potenially malignant and are curable if diagnosed and treated early. The patients may have associated congenital anomalies. This patient has spina bifida cystica in lower sacrum. Brief review of related literature is included... |
In this study I assesed the efficacy and tolerance of cyclosporin A (CyA) in the treatment of steroid-dependent MCNS (minimal change nephrotic cyndrome) and steroid resistant FSGS (focal segmental glomerulosclerosis). The results showed, that 1) CyA was effective in sustaining a remission in steroid-dependent MCNS without prednisone treatment, and therefore, could be an alternative therapeutic choice. 2) After discontinuation of CyA, relapses reccurred... |
Poststreptococcal reactive arthritis/arthralgia is characterized as an evidence of group A beta hemolytic streptococcal infection and does not fulfill the modified Jones criteria for a diagnosis of acute rheumatic fever. We had used to meet the patients with incomplete acute rheumatic fever who had more than 3 items of minor Jones criteria, or arthralgia or arthritis with one or two... |
A clinical study was made on 12 cases of neonatal Group Bβ-hemolytic streptococcal(=GBS)meningitis, who were admitted to the department of Pediatrics, Dong-San Hospital, Keimyung University during the period of 3 years from Aug 1989 to Jul 1992. The following results were obtained 1) GBS was cultured in 12 cases(57.1%) among 21 neonatal meningitis admitted during the same period. 2) Male and female ratio... |
Virus associated hemophagocytic syndrome, class II histiocytoses, characterized by high fever, severe constitutional symptoms, abnormal liver function and coagulation, peripheral blood pancytopenia and histiocytic hyperplasis with prominent hemophagocytosis in bone marrow and lymph nodes has been reported and associated with active viral infection. It is non-malignant and reversible. It must be differentiated from histiocytic medullary reticuosis because of the inappropriateness... |
Thirty one infants of diabetic mothers(IDM) who had been admitted in Neonatal Intensive Care Unit at Chonnam University Hospital from January 1987 to July 1991 were studied for evaluation of their perinatal outcome and prognosis. The results were as follws; 1) The distribution of diabetic mothers according to modified White뭩 classification was GD & class A in 12 cases (38.7%), B in... |