We experenced a case of cystic pancreatoblastoma associated with congenital hemihypertrophy in a 4months old male. The mass was located on the anterior side of pancreatic head without any connection to the pancreas. After exision of cystic pancreatoblastoma, chemotherapy(FAM regimen) was performed 15 times due to capsular tumor invasion. Until this time there was no drug side effect and metastasis.... |
Purpose : Neonatal seizure is usually the presentation of underlying neurologic dysfunction rather than a disease and have high mortality and morbidity as sequalae. Therefore, the parameter predicting the neurologic prognosis is necessary. This study was performed to identify the usefulness of clinical scoring as predictor in neonatal seizure by analyzing outcomes. Methods : From 1982 to 1994, 101 neonates were admitted to Neonatal Intensive... |
Purpose : The newborn period is a time of rapid physiological change in the gastrointestinal tract, when adaptation from a state of parenteral nutrition to the demands of enteral feeding take place. little is known about the intestinal vascular responses that accompany intestinal adaptive changes, although there is much speculation about the relationship between entreral feeding, alterations in gut perfusion,... |
Hypothalamic hamartoma is a congenital anomaly presenting precocious puberty, gelastic seizure and mental retardation. Other possible accompanying anomalies are cleft palate, tetralogy of Fallot, skeletal malformation and anal malformation. But the patient describing here manifested gelastic seizure only with isosignal mass of 1.5cm in diameter situated just behind optic chiasm in T1WI and T2WI of MRI. His EEG showed multifocal... |
Purpose : Mucormycosis is an opportunistic fungal infection caused by one of the ubiquitous fungi of the order Mucorales, occurring almost exclusively in immunocompromised hosts such as patients with diabetes, leukemia and lymphoma. Recently the incidence of mucormycosis is rising associated with the increasing predisposing factors such as cytotoxic drugs and immunosuppressive agents. Though mucormycosis is frequently fatal, there has... |
Purpose : Since 1990 upper intestinal endoscopy has become frequently used tools for the diagnosis and treatment of a variety of upper intestinal tract disease. The purposes of this study was to assess the usefulness of small electronic endoscope in pediatric patients. Methods : Upper intestinal endoscopy was performed in 104 cases of children for 15 months using electronic endoscope (Olympus... |
Purpose : Pancreatitis in children is not common but can be associated with severe morbidity rates. Early prognostic evaluation of acute pancreatitis assists in selecting those patients who should be closely monitored. We studied the clinical characteristics and prognostic factors in patients with acute pancreatitis. Methods : Twenty five children with pancreatitis were included ranging in age from 1.8 to 14.9... |
Multicystic dysplastic kidney is the most frequent cause of abdominal mass in the neonate. It is frequently associated with contralateral genitourinary tract abnormalities and the most common abnormality is vesicoureteral reflux. Renal artery aneurysm is very rare in the children. Furthermore the case associated with the renal artery aneurysm has not been reported yet. We experienced a case of the left... |
Purpose : To assess the differences of clinical features according to various karyotypes in Turner syndrome. Methods : Subjected to study were 45 patients with Turner syndrome, including 5 newborns, from March 1974 to April 1994. They were divided into 3 groups according to karyotypes: 45,X, mosaicism, and structural aberration; and the clinical features were compared. Also structural aberration groups, 46,XXp-and... |
Since Brown first descirbed the eosinophilic granuloma in 1960, 45 cases of eosinophilic cystitis have been reported in the literature, including 10 pediatric cases. In Korea, only four adult cases were reported. Eosinophilic cystitis is characterized by severe infiltration o eosinophils into the mucosa, submucosa and muscularis of bladder wall which causes irritative voiding symptoms. This lesions have been frequently... |
Pneumoperitoneum in the newborn is usually a surgical emergency resulting from gastroint-estinal perforation. However, pneumoperitoneum can occur secondary to a pulmonary air leak which progresses from the mediastinum to the retroperitoneum by way of perivascular spaces and then to the peritoneum following rupture. We have observed this phenomenon in three ventilated newborn. A brief review of the related literatures was... |
Purpose : Drining of Nitrate-contaminated water has been the most common cause of acquired methemoglobinemia. We had cyanotic infant diagnosed as methemoglobinemia caused by feeding with powdered formula mixed with nitrate-contaminated ground water. Methods : We had done epidemiologic investigation to identify the cause of an infant's methemoglobinemia. Analysis of ground water and blood test of involved family members and neighbors... |
Familial erythrophagocytic lymphohistiocytosis(FEL) is an uncommon disorder characterized by multi-organ infiltration with phagocytic histiocytes and macrophages. It is a familial discorder presenting during infancy or young childhood with fever, hepartosplenomegaly, pancytopenia, bleeding diathesis, hypertriglyceridemia and neurologic manifestations. The course of the disease is extremely lethal and diagnosis of the disease during lifetime is very difficult. Exact diagnosis can only be... |
Purpose : Infection is a major complication in patients with malignant disease. This study was performed to identify the causes and the etiologic agents of febrile infections and to characterize the clinical courses including the response to antimicrobial agents inpediatric cancer patients. Methods : This study reviewed 274 febrile episodes occurring in 163 children with neoplastic disease which were indentified prospectively... |
Purpose : Cerebrovascular disease in children is more common than was once recognized and the etiology and prognosis of the disease in children are quite different from those of adults. Recently according to medical reports and clinical experiences, the incidence of childhood cerebrovascular disease is increasing in Korea. To clarify the clinical features of childhood cerebrovascular disease and to analyze its... |
Purpose : Paranasal sinusitis, easily overlooked owing to it's benign symptomatology, should be considered when the upper respiratory tract infction persists longer than seven days. Without early recognition and appropriate management, the disease will take a chronic course and the incidence of complications will be increased. The significance of paranasal sinusitis among Korean children has not appropriately been appreciated; therefore,... |
We report three cases of cystinuria, presenting with urinary stones. A 2-year-old girl presented with urinary difficulty, hematuria, dysuria of sudden onset, and her 7-month-old younger brother also was presented with urinary difficulty, irritability on urination & stone passage. Other 6-month-old boy was admitted due to sudden onset anuria. They had radioopague renal & ureter stones and stone analysis revealed mixed... |
Purpose : AHC characterized by sudden onset of gross hematuria, dysuria and frequency oc curs in children and young adults as a self-limited disease that should be differentiated from se rious renal disorders. We have performed this study to establish the cause and characterize the clinical features of this illness in Korean children. Methods : 19 cases collected prospectively for 30... |
The cystic disease of the kidney include a heterogeneous group of developmental, hereditary, and acquired disorders. Based on extensive microdissection studies, Potter concluded all renal cystic diseases could be categorized into four types. We have experienced 5 cases of cystic kidney disease which were confirmed by aoutopsy and classified as Type I, Type II, Type III, Boderline between types II and... |
Purpose : A Study was undertaken to assess the family background of abandonment and clinical and social aspects in an institute for foreign adoption. The results were compared to the previous two reports from the institute. Method : A sample of 1,728 children from 1987 to 1992 formed the subjects of the study by the medical records in the institution. Results :... |
Purpose : Recurrent abdominal pain (RAP) in children is similar to nonulcer dyspepsia of adult. Recently, microscopic inflammation and Helicobacter pylori (H. pylori) infection have been suggested as possible causes of nonulcer dyspepsia in adults. The aim was to know the clinical significance of encoscopic findings and H. pylori infection in children with RAP. Methods : 128 children with RAP underwent... |
High dose intravenous gammaglobuline (IVIG) therapy is effective in some of the autoimmune diseases. Although the exact mechamism of action of IVIG is uncertain, the action as a neutralizing antibody against unknown etiologic agents, the action of blocking of Fc receptors of effector cells, or the action as a antiidiotypic antibody are suggested. We report a case of 12 year old... |
Transfer of large quantities of fetal blood across the placental barrier to the maternal circulation is a rare occurrence which results in severe anemia in the newborn infants. This phenomenon is believed to occur most often during labor and delivery and apparently, is more frequent when abnormal obstetric conditions are present. However, fetal erythrocytes have been identified in the naternal... |
To elucidate the clinical characteristics-especially its clinical pattern and outcome of chidren with encephalopathy, we carried out a retrospective review on medical records of 45 patients who were intially diagnosed as encephalopathy except mumps meningitis, Reye's syndome and neonatal hypoxic ischemic encephalopathy at the Department of pediatrics, Hanyang University children's hospital from January 1986 to February 1994. The results were summerized... |
To elucidate the clinical availability of FNAB, we reviewed retrospectively medical records of 30 patients who had infiltrative pulmonary lesion or pulmonary nodule of mediastinal mass, and got percutaneous fine needle aspiration biopsy using Wescott needle, 20~22 gauge, at the department of Pediatrics, Hanyang university hospital from July, 1986 to June, 1993. The obtained results were as follow: 1) There were 30... |
Immunosuppressive therapy based on the use of antilymphocyte globulin (ALG) has become standard therapy for patients with aplastic anemia who are not eligible for bone marrow transplantation. In this study, T cell subsets before and after ALG therapy, hematologic responses, complications and prognostic factors were analysed. Eleven (42%) out of twenty-six patients treated with ALG showed response, but... |
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... |
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... |
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.... |
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... |
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