BACKGROUND: Childhood myelodysplastic syndrome(MDS) is a heterogenous disease complex which has characteristics of cytopenia in one or more hemopoietic cell lines in peripheral blood and of dysmorphisms of hemopoietic precursors in bone marrow, but quite different from adult one. We experienced 22 patients with childhood myelodysplastic syndrome during 9 years from 1987 to 1995 in the Department of Pediatrics, Inje University College of Medicine, Pusan Paik Hospital, Pusan, Korea and performed a clinical study about them to analyze the clinical and hematological features and treatment outcome retrospectively. METHOD: We analyzed their clinical features including age and sex distribution, chief complaints and physical findings on first admission, distribution of subtypes, hematologic features including initial hemoglobin, total WBC and platelet count, and treatment outcome including leukmic transformation and prognostic scores. RESULTS: 1) Of the total 22 patients, 10 were in 0-4 years of age on first admission, 8 in 5-9, and 4 above 10 years of age. The male : female sex ratio was 2.1 : 1. 2) Of the 22 patients, chief complaints on first admission were bleeding tendency in 9 patients(40.9%), pallor in 8(36.4%) and fever in 3(13.6%), in order, and physical findings on first admission were purpura or ecchymoses in 17(77.3%), anemia in 15(68.2%) and hepatosplenomegaly in 8(36.4%), in order. 3) Hematologic findings on first admission were as follows : hemoglobin levels were below 3 g/dl in 1 patient(4.5%), 3-6 g/dl in 10(45.5%), and 6-9 g/dl in 11(50.0%). Initial WBC counts were below 5,000/mm3 in 13 patients(59.1%), 5,000-10,000/mm3 in 5(22.7%) and above 10,000/mm3 in 4(18.2%). Initial platelet counts were below 20,000/mm3 in 10(45.5%), 20,000-50,000/mm3 in 5(22.7%), 50,000-100,000/mm3 in 5(22.7%) and above 100,000/mm3 in 2(9.1%). 4) Of the 22 patients, 12 patients(54.6%) were RA type, 1(4.6%) RAS, and 3(13.6%) RAEB, RAEB-T and JCML types, respectively. 5) According to prognostic scores by Mufti et al(1986), none were in 'good' group, 17 patients(89.5%) in 'intermediate' group with 39.5 months of mean duration of survival(range 4-95 months) and 2(10.5%) in 'poor' group with 18 months of mean duration of surviral(range 17-19) until the last follow-up. However, the subtypes and clinical status seemed not to be related to the prognostic scores. 6) Sixteen patients were treated with low dose cytosine arabinoside(10 mg/m2/12hrs), of whom 7 patients gained long-standing event-free survival, whose treatment regimen was changed to oral 6-TG about 2 years later. All 3 of JCML were treated with A-Triple-V regimen, one of whom was died of sepsis, one was transformed into AML and died of sepsis, while the remained one gained long-standig event-free survival (62 months). 7) Leukemic transformation into AML occured in 7 patients(RA 1, RAEB 2, RAEB-T 3, JCML 1), 6 of whom were dead, while one gained long-standing event-free survival of 34 months. CONCLUSION: We concluded that RA was the most dominant type among our patients, and the frequency to transform into AML was 31,8%, and 31.9% of the patients had long-term survival, and that reliability of prognostic scoring system by Mufti et al(1986) was not high.
Adult ; Anemia ; Anemia, Refractory, with Excess of Blasts ; Bone Marrow ; Busan ; Cell Line ; Cytosine ; Disease-Free Survival ; Ecchymosis ; Female ; Fever ; Follow-Up Studies ; Hemorrhage ; Humans ; Korea ; Male ; Myelodysplastic Syndromes* ; Pallor ; Pediatrics ; Platelet Count ; Purpura ; Retrospective Studies ; Sepsis ; Sex Distribution ; Sex Ratio ; Treatment Outcome

No abstract available.
Peptic Ulcer* ; Retrospective Studies*

No abstract available.
Chorea* ; Humans

No abstract available.
Child* ; Humans

The aims of the Tharies replacement are to preserve bone stock and provide a more physiological and anatomical replacement using eccentic shells with the hope that long term durability will be improved. 8 cases of THARIES replacement performed between 1981 and 1983 at Hanyang UniversityHospital were reviewed and analyzed by radiography and U.C.L.A. hip rating system. Follow-up ranged from 34 to 54 months and averaged 43.3 months. The result was relatively good in 6 cases except two cases of aseptic loosening without further progression.
Arthroplasty ; Follow-Up Studies ; Hip ; Hope ; Radiography

A patient with primary adenocarcinoma of Bartholin's gland is reported and the literatures relevant to this disease reviewed. Not only this disease is very rare but also primary carcinomas of Bartholin's gland are misdiagnosed as cysts or abscesses in half of the cases, leading to considerable delay in diagnosis. And so, It was wasted long time before definitive therapy. However, because of a different clinical behavior, cancer of the Bartholin's gland should be distinguished from other vulvar carcinomas. Histologically, squamous cell carcinoma and adenocarcinoma are the most common. Virtually all histologic types of Bartholin's gland carcinoma metastasize to lymph node, bone, lung and liver in distant sites. The authors data and a review of the literature support the concept that radical vulvectomy with or without bilateral inguina1-femoral lymphadenectomy is required. On the other hand, except primary radiation treatment for small or medium sized cancers, the results obtained by radiation therapy in carcinoma of the vulva including Bartholin's gland are generally discouraging. A role for postoperative adjuvant radiation therapy suggests because of high incidence of positive inguinal-femoral lymph nodes. In the near time, natural history and biological behavior of Bartholin's gland cancer must be disclosed in detail. And also optimal treatment modality and prognostic factors shall be determine.
Abscess ; Adenocarcinoma* ; Carcinoma, Squamous Cell ; Diagnosis ; Hand ; Humans ; Incidence ; Liver ; Lung ; Lymph Node Excision ; Lymph Nodes ; Natural History ; Vulva

Fourteen total cystectomy specimens with primary carcinoma of urinary bladder were investigated to illustrate the overall prevalence and nature of mucosal changes adjacent to the overt carcinoma and to clarify the distribution pattern and extent of carcinoma in situ in regard with multiplicity, histological type and depth of invasion of the macroscopically visible tumor mass. Of 14 cases subjected to this study were 12 cases of transitional cell carcinoma of varying degree of differentiation and invasion, one adenocarcinoma, and the another one with no grossly detectable tumor but history of previous cystoscopic removal of papillary transitional cell carcinoma. Each specimen was processed by a histotopographic technique developed by authors, an easy access to reconstruct the mucosal changes using reconstruction paper. For the histological grading and staging of the overt carcinomas, Ash's and Collins' classifications were applied, respectively. Carcinoma in situ (CIS) was found in 10 out of 11 cases with overt urothelial carcinoma of urinary bladder and one case with no grossly visible neoplasm, in which cystoscopic removal of papillary carcinoma was carried out previously. Distribution pattern of CIS was mostly circular (group I) or arborizing (group II) , located around the overt carcinoma within 0.5 to 2.0cm., but not beyond 3.0cm. from its margin. Correlation between multiplicity of overt carcinomas and presence of CIS was evident together with irregularity of its distribution, but no significance was found with gross appearance, histological grading or with staging.
Adenocarcinoma ; Carcinoma in Situ* ; Carcinoma, Papillary ; Carcinoma, Transitional Cell ; Classification ; Cystectomy ; Mucous Membrane* ; Prevalence ; Urinary Bladder*

No abstract available.

Benign symmetric lipomatosis is characterised by diffuse symmetric deposits of adipose tissue, predominantly on the neck, shoulder, back and upper extremities. We report a case of benign symmetric lipomatosis in a 61-year-old-man, which was first noted one year previously. It began growing rapidly about 3 months ago and was associated with alcoholic liver disease. Histopathological findings showed that the reticular dermis had been replaced by normal uncapsulated mature fat cells that were slightly increased in number in the fibrous connective tissue. He was treated with theophylline 300mg/day for two months.
Adipocytes ; Adipose Tissue ; Connective Tissue ; Dermis ; Lipomatosis* ; Liver Diseases, Alcoholic ; Neck ; Shoulder ; Theophylline ; Upper Extremity

No abstract available.
Burns* ; Hand* ; Humans ; Infant*