No abstract available.
Precursor Cell Lymphoblastic Leukemia-Lymphoma*

No abstract available.
Wilms Tumor*

The aspergillus tracheobronchitis is distinctive manifestation of invasive aspergillosis, in which infection is limited completely or predominantly to the tracheobronchial tree. It accounts for about 7 to 10 percent of cases of invasive disease. Grossly, such disease may take the mucosal exudate and obstruct partially the airway lumen or completely the occlusive mucous/fungus plugs. Microscopically, the superficial portion of the airway wall is acutely inflamed and contain fungal hyphae. However, infection is often limited to the mucosa. We report a case of aspergillus tracheobrochits in a 54 year-old man who presented cough, progressive dyspnea with wheezing, and mucus plug. Bronchoscopy showed mucosal exudate and plug.Bronchoscopic biopsy showed aspergillus hyphae and inflammation in the mucosa. He was successfully treated with itraconazole.
Aspergillosis ; Aspergillus* ; Biopsy ; Bronchoscopy ; Cough ; Dyspnea ; Exudates and Transudates ; Humans ; Hyphae ; Inflammation ; Itraconazole ; Mucous Membrane ; Mucus ; Respiratory Sounds ; Trees

No abstract available.
Child* ; Humans

PURPOSE: Cepharanthin (R) has been reported to improve the symptoms of intractable or steroid-resistant chronic idiopathic thrombocytopenic purpura (ITP). We report the clinical efficacy of oral high dose Cepharanthin (R) in refractory chronic ITP. METHODS: We analyzed eleven patients who were diagnosed as chronic ITP at the Department of Pediatrics, Seoul National University Hospital from January, 2002 to February, 2004. After informed consent patients with platelet count below 20, 000/mm3 were treated with Cepharanthin (R) at the initial dose of 5 gm/day. The doses were increased up to 15 gm/day if there was no response. For the purposes of this study, complete remission was defined as an elevation of the platelet count > or =50, 000/mm3 from the baseline after 4 weeks of treatment. Partial remission was defined as an elevation of the platelet count 20, 000/mm3 and < = or50, 000/mm3 from the baseline. RESULTS: Seven boys and four girls with a median age of 10 were enrolled. Two to four weeks after the initiation of this therapy, 4 patients showed their platelet counts over 50, 000/mm3 1 patients reached partial remission state, 6 patients had no response. Side effects of Cepharanthin (R) were not observed in all patients. CONCLUSION: We suggest that the oral administration of Cepharanthin (R) could be a beneficial and a safe treatment strategy for the refractory chronic ITP patients. Further studies should be performed to elucidate the mechanism of responses.
Administration, Oral ; Child* ; Female ; Humans ; Informed Consent ; Pediatrics ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic* ; Seoul

Stickler syndrome is an autosomal dominant disorder of connective tissue with a wide range of expressivity and incomplete penetrance which is called hereditary progressive arthro-ophthalmopathy. Affected neohates may present with the Pierre-Robin syndrome, progressive myopia, retinal detachment, flat face, hypertelorism, progressive arthritis. Early recognition of the syndrome is important, not only for genetic counselling but also to offer a more precise prognosis and proper treatment of many serious disorders that may occur in affected children. We experienced a case of Stickler syndrome with large eyeballs in a 3-day-old female baby who showed Pierre-Robin anomaly, flat face, hypertelorism, epicanthal folds, long philtrum, micrognathia, deft palate, high congenital myopia, chorioretinal degeneration, thin habitus and hyperextensible joints.
Arthritis ; Child ; Connective Tissue ; Female ; Humans ; Hypertelorism ; Joints ; Lip ; Myopia ; Myopia, Degenerative ; Palate ; Penetrance ; Pierre Robin Syndrome ; Prognosis ; Retinal Detachment

Pulmonary aspergillosis is classified as a saprophytic, allergic, and invasive disease. Chronic necrotizing pulmonary aspergillosis is categorized as an invasive pulmonary aspergillosis. Most invasive pulmonary aspergillosis have acute and toxic clinical features but chronic necrotizing pulmonary aspergillosis is characterized by a sub-acute infection, most commonly seen in patients with altered local defense system from preexisting pulmonary disease of in mild immunocompromised patients. Pulmonary artery aneurysm due to this infection is termed as a mycotic aneurysm, etiology of which are tuberculosis, syphilis, bacteria and fungus. We report a case chronic necrotizing pulmonary aspergillosis complicating pulmonary aneurysm is a 62 year-old man who was presented with cough, sputum, and fever. Chest radiographs showed a rapid, progressive cavitary lesion and pulmonary artery aneurysm. Angioinvastion of aspergillus was revealed by pathology after operative removal of left upper lobe containing the pulmonary artery aneurysm. He was treated with itraconazole.
Aneurysm* ; Aneurysm, Infected ; Aspergillus ; Bacteria ; Cough ; Fever ; Fungi ; Humans ; Immunocompromised Host ; Invasive Pulmonary Aspergillosis* ; Itraconazole ; Lung Diseases ; Pathology ; Pulmonary Artery* ; Pulmonary Aspergillosis ; Radiography, Thoracic ; Sputum ; Syphilis ; Tuberculosis

XX male has a male phenotype with testes or gonads of testicular type and a female chromosomal constitution of 46, XX with no evidence of either ovarian tissue or female genital organs. Generally, they have normal male genitalia and all are infertile. We experienced a neonate with anophthalmia, hypospadia, small penis, and normal testes, whose chromosomal analysis demonstrated 46, XX. Polymerase chain reaction revealed the existence of a sex-determining region of Y (SRY). These findings suggest that the translation of an SRY on the X chromosome led to the development of a male phenotype. We report the case with a review of the related literature.
46, XX Testicular Disorders of Sex Development* ; Anophthalmos ; Constitution and Bylaws ; Female ; Genitalia, Female ; Genitalia, Male ; Gonads ; Humans ; Hypospadias ; Infant, Newborn ; Male ; Penis ; Phenotype ; Polymerase Chain Reaction ; Testis ; X Chromosome

We experienced a case of spondylothoracic dysplasia in a 1 day old male who has short neck with limitation of movement, asymmetric thorax, marked scoliosis and a systolic heart murmur. He was born with normal weight and full term. The history of his family and mother is not specific. On x-ray, multiple hemivertebrae in thorax, spina bifida and maldevelopmental thoacic cavity were detected. On echocardiogram, he had small ASD and PDA.
Heart Defects, Congenital* ; Heart Murmurs ; Humans ; Male ; Mothers ; Neck ; Scoliosis ; Spinal Dysraphism ; Thorax

The failure to improve the five-year survival rate of cancer patients, from one in three in the 1960s to one in two in the 1970s, stimulated awareness of the importance of primary prevention of cancer. Korean investigators carried out extensive long-term anticarcinogenicity experiments with 2000 newborn mice to investigate whether Panax ginseng C.A. Meyer inhibited carcinogenesis induced by several chemical carcinogens in 1978. There was a 22% decrease (p<0.05) in the incidence of urethane induced lung adenoma by the combined use of red ginseng extract. In the group sacrificed at 56 weeks after the treatment with aflatoxin B1, the incidence of hepatoma significantly decreased to 75% by the addition of red ginseng extract (p<0.05). The result showed that natural products can provide hope for human cancer prevention. By the newly established '9 week medium-term anticarcinogenicity test model of lung tumors in mice' (Yun's model), we confirmed significant anticarcinogenic effects of powders and extracts of the 6- yr-old dried fresh ginseng, 5- and 6-yr old white ginsengs, and 4-, 5-, and 6-yr old red ginseng. We also demonstrated that the anticarcinogencity of ginseng was more prominent in aged or heat treated extracts of ginseng and red ginseng made by steaming. To investigate the active components for cancer prevention, several fractions of 6-yr old fresh ginseng and red ginseng, four semi-synthetic ginsenoside Rh1, Rh2, Rg3 and Rg5, major saponin components in red ginseng, were prepared. Among the ginsenosides, Rg3 and Rg5 showed statistically significant reduction of lung tumor incidence and Rh2 had a tendency of decreasing the incidence. Ginsenoside Rg3, Rg5 and Rh2 were found to be active anticarcinogenic compounds. Rg3, Rg5 and Rh2 are active components in red ginseng, and they prevent cancer either singularly or synergistically.
Animal ; *Anticarcinogenic Agents ; Disease Models, Animal ; Fractionation ; Human ; Korea ; Mice ; Molecular Structure ; *Panax/chemistry/growth & development ; Plant Extracts/analysis ; Time Factors