Adult Wilms' tumor, unlike that of childhood, is a rare disease. Microscopically, the tumor is fundamentally characterized by triplastic embryonal renal tumor composed of variable amounts of metanephric blastema and its epithelial and stromal derivatives but rarely a small group of tumors composed virtually entirely of differentiated epithelial derivatives, the abundance of tubular structures. These monomorphous epithelial type of Wilms' tumor tended to have an early onset and benign course. Grossly, classic Wilms' tumor is a solid tumor, but very rarely shows cystic change and may lead to misinterpretation as a polycystic kidney or multicystic nephroma. Here, we reports a case of primary renal tumor, grossly very similar to a multicystic kidney but histologically represent a tubular monomorphous epithelial variant of Wilms' tumor occured in 63 year old male adult.
Child ; Adult ; Male ; Female ; Humans

No abstract available.
Cheek* ; Rhabdomyosarcoma*

No abstract available.
Acne Conglobata* ; Acne Vulgaris* ; Steroids*

Twenty-two cases of idiopathic myocardiopathy were observed during the period of 1962 to 1971 and follow up clinical study was made in 5 cases. The criteria of diagnosis was based mainly on exclusive diagnosis in etiology unknown marked cardiomegaly. 1) Male and female ratio of idiopathic myocardiopathy was 1.1:1 and age of onset were distributed from first decade to fifth decade with similar number of cases. 2) Cardinal symptoms were dyspnea, palpitation, chest pain and cough. Common physical findings were protodiastolic gallop (in 2 cases), moist rales (in 3 cases) and hepatomegaly(in 3 cases). 3) Laboratory findings were normal except elevated T.T.T. in one case. 4) Electrocardiogram were abnormal in all cases. There were 3 cases of left ventricular hypertrophy, 3 cases of non-specific ST-T changes and one case of first degree A-V block, intraventricular conduction defect, abnormal Q wave and low valtage, respectively. 5) After medical treatment, symptoms were improved in 4 cases but heart size was reduced in only one case and E.C.G. abnormalities were unchanged in all cases.
Age of Onset ; Cardiomegaly ; Cardiomyopathies* ; Chest Pain ; Cough ; Diagnosis ; Dyspnea ; Electrocardiography ; Female ; Follow-Up Studies ; Heart ; Humans ; Hypertrophy, Left Ventricular ; Male ; Respiratory Sounds

Letterer-Siwe disease occurred in a 3 year 7 month old male child showing severe emaciation, hepatosplenomegaly, otitis media, hemorrhagic and impetiginous crusted patches on the body, and seborrheic dermatitis like skin rashes in the scalp. Also showed are severe anemia, thrombocytopenia, bronchopneumonia and multiple bony defects in skull. The child expired in a month after admission.
Anemia ; Bronchopneumonia ; Child ; Dermatitis, Seborrheic ; Emaciation ; Exanthema ; Histiocytosis, Langerhans-Cell* ; Humans ; Infant ; Male ; Otitis Media ; Scalp ; Skull ; Thrombocytopenia

One hundred eighty-six cases of ovarian common "epithelial" tumor including 28 endometriosis were reviewed, from the pathology file of the department of pathology of the National Medical Center during a period of 8 years and 6 months from January 1976 to June 1984. The study consisted of 14 cases(77.4%) of benign tumor including 28 cases of ovarian endometriosis, and 21 cases(11.3%) of borderline malignancy and 21 cases(11.3%) of invasive malignant tumors. Among benign common "epithelial" tumors, mucinous cystadenoma(69 cases) was the most frequent tumor, followed by serous cystadenoma (42 cases) and Brenner tumor (5 cases). Of borderline tumor, borderline mucinous tumor (18 cases) was more frequent than serous tumor (3 cases). Serous cystadenocarcinoma(9 cases) was the most frequent malignant tumor, followed by mucinous cystadenocarcinoma(6 cases), endometrioid carcinoma(3 cases) and undifferentiated carcinoma(3 cases). Most ovarian common "epithelial" tumors were prevalent during the reproductive age, but malignant tumors were more prevalent after the age of 40. Although right side was more frequently found (85 vs. 67) there is no significant site predilection. Bilaterality was the most common in undifferentiated carcinoma(66.7%), followed by serous cystadenocarcinoma(44.4%) and Brenner tumor(40%). The size of tumor tended to be larger in malignant tumor than benign. Mucinous tumor were usually larger than serous tumor. There were 7 intrauterine and 1 ectopic tubal prognancy which were associated with ovarian common "epithelial" tumors. Three cases of tumors were excised during Cesarian section. In summary these findings are comparable to other reports in Korea and English literatures. Histologic analysis of borderline and malignant epithelial tumor was done to emphasize their biologic behavor. Further study is required to compare their histological grading and clinical staging with thier survival rate.

Blue Rubber Bleb Nevus syndrome, or Bean's syndrome is a very rare disease characterized by an association of 1) hemangioma cutis in which the nevi feel like rubber blebs; 2) hemangiomas of the digestive organs; 3) iron-deficiency anemia due to hemorrhage from the digestive tract. Sometimes, the bleeding is so severe and massive that the patient needs blood transfusions and/or emergency operations. We present a 29-year-old man with Blue Rubber Bleb Nevus syndrome. This patient was admitted to out hospital due to recently aggrevated dizziness and intermittent rectal bleeding. Multiple bluish tumors were noted on the palm, sole and glans penis. Laboratory examination revealed severe iron-deficiency anemia. Barium studies revealed multiple polypoid masses in the stomach, small bowel and colorectum. On esophagogastroduodenoscopy and colonoscopy, three or four dozens of hemangiomas with variable size and shape were noted in the stomach, duodenum at or around the ampulla of Vater and colorectum. This patient was of particular interest because rectal bleeding occurred from rectal lesion which protruded out of anus and caused bleeding during defecation, and which showed as a 2.0 cm Yamada type III polypoid lesion. Endoscopic ultrasonography confirmed us that this lesion was confined to the mucosa and submucosa without extension into muscle layer. Endoscopic polypectomy of this lesion was performed because which considered as the main focus of current rectal bleeding. He was discharged without any complication and 4 months later, he was well without rectal bleeding and his hemoglobin level increased upto 13.5 g/dl. To our knowledge, this is the first case of Blue Rubber Bleb Nevus syndrome in Korea.
Adult ; Ampulla of Vater ; Anal Canal ; Anemia, Iron-Deficiency ; Barium ; Blister* ; Blood Transfusion ; Colonoscopy ; Defecation ; Dizziness ; Duodenum ; Emergencies ; Endoscopy, Digestive System ; Endosonography ; Gastrointestinal Tract ; Hemangioma ; Hemorrhage ; Humans ; Korea ; Male ; Mucous Membrane ; Nevus* ; Penis ; Rare Diseases ; Rubber* ; Stomach

No abstract available.
Postoperative Complications*

Chlinnical observation was conducted on 10 cases of infants of diabetic mother at II Sin Woman's Hopital and the Busan National University Hospital during 19 months from Jan. 1977 to Jul. 1978. The results were summarized as follows : 1. Frequency of diabetic mothers was 0.094% and most of them were in 3rd decade of age. 5 mothers treated, two of them treated irregularly, and five not treated during pregnacy had the mean duration of 3 years from onset of diabetic symptoms to delivery. 2. Diabetic mother whose mean gravidity was 3.6 had histories of toxemia(50%), natural abortions or stillbirths(19%), and I case of polyhydramniosis and pyelonephritis respectively. 3. Among 10 cases of infants of diabetic mothers (5 full terms and 5 preterms), four large for gestational age, three appropriate for gestational age, and three small for gestational age, seven of them were delivered with vaginal and three with C-S deliveries. 4. Among 10 cases of infants of diabetic mothers, two of three infants with hypoglycemia had Apgar score below 6 at 1 minute and died soon after birth, and I case showed hypoglycemic symptoms such as tremor, sweating and so on. Hyperbilirubinemia ranging from 10 to 18 mg/dl in 3 cases, RDS 2 cases, and congenital anomaly 3 cases were also observed.
Apgar Score ; Busan ; Gestational Age ; Gravidity ; Humans ; Hyperbilirubinemia ; Hypoglycemia ; Infant* ; Mothers* ; Parturition ; Pyelonephritis ; Sweat ; Sweating ; Tremor

Bone marrow transplantation has become an accepted treatment for malignancy(particulary leukemia and lymphoma), aplastic anemia, and certain inborn errors of metabolism. In addition to the problem of severe, prolonged myelosuppression, bone marrow transplantation is associated with several unusual complications. Among the complications such as GVHD, graft rejection, interstitial pneumonia and veno-occlusive disease, involvement of the gastrointestinal tract by GVHD is associated with high graft failure and mortality. Intestinal GVHD is usually manifest clinically as voluminous secretory diarrhea accompanied by abdominal cramping, ileus, nutritional depletion, and, at times, hemorrhage. We experienced a case of severe intestinal GVHD after allogeneic marrow transplantation for treatment of severe aplastic anemia. He received bone marrow from his elder sister, HLA-matched multiparous woman and suffered from large amount of watery diarrhea with skin rash 34 days after transplantation. 1n spite of prednisolone therapy the symptom was progressed. After sigmoidoscopic mucosal biopsy, intestinal GVHD was confirmed and we tried methylprednisolone pulse therapy. Skin lesion was improved but the amount of diarrhea was increased with intermittent abdominal cramping. We tried ALG(anti-lymphocyte globulin) and conservative management but the patient did not respond the therapy. He succumbed to pneumonia and acute respiratory insufficiency complicated with GVHD, 70days after transplantation.
Anemia, Aplastic* ; Biopsy ; Bone Marrow Transplantation* ; Bone Marrow* ; Colic ; Diarrhea ; Exanthema ; Female ; Gastrointestinal Tract ; Graft Rejection ; Hemorrhage ; Humans ; Ileus ; Leukemia ; Lung Diseases, Interstitial ; Metabolism, Inborn Errors ; Methylprednisolone ; Mortality ; Pneumonia ; Prednisolone ; Respiratory Insufficiency ; Siblings ; Skin ; Transplants