Sjogren's syndrome has been considered to be an autoimmune disease affecting various organs including salivary and lacrimal glands. It occurs most commonly in middle-aged women, and less than 20 cases with primary Sjogren's syndrome have been reported in children. An 11-yim-old boy presented with recurrent annular erythema on the face that had been present for 2 years. A schirmers test showed a positive result. ANA was detected at a dilution of 1:640. Anti-Ro/SSA and anti-La/SSB antibodies were also detected. On histological examination, lymphocytes were infiltrated in the periappendegeal areas as well as the papillary and reticular dernis. The skin findimg is uncommon in children, but has become a characteristic feature of childhood Sjogrens syndrome with anti-Ro and/or anti-La antibodies.
Antibodies ; Autoimmune Diseases ; Child ; Erythema* ; Female ; Humans ; Lacrimal Apparatus ; Lymphocytes ; Male ; Sjogren's Syndrome* ; Skin

No abstract available.
Orchiectomy* ; Testis*

No abstract available.
Factor VIII* ; Prevalence*

Purpose: The purpose of this study was to evaluate the clinical and radiological results of total hip arthroplasty using a proximal modular femoral stem in patients who had secondary coxarthrosis associated with a deformed femur. Materials and Methods: Forty-two patients (45 hips) with secondary coxarthrosis were evaluated after primary total hip arthroplasty using an S-ROM proximal modular femoral stem, between January 2001 and March 2004. The average follow-up was 44 months (range, 24 to 60 months). The mean age of the patients was 48.5 years old and there was a predominance of female patients (65.5%). The preoperative diagnoses included 26 cases of developmental dysplasia of the hip, 13 cases of sequalae of LCP, 2 cases of epiphyseal dysplasia, 3 cases of sequalae of pyogenic arthritis, and 1 case of congenital coxa vara. Results: The average Harris hip score improved from 52.2 points to 85.5 points. All the femoral stems demosntrated stable fixation, which included 37 cases by bony ingrowth and 8 cases by stable fibrous ingrowth. Neither osteolysis nor progressive radiolucent lines around the femoral stems were found at the latest follow-up. Postoperative complications included: 2 cases of hip dislocations, 1 case of periprosthetic fracture, 1 case of infected cup loosening, and 1 case of sciatic nerve palsy. Overall, forty-one hips (91.9%) exhibited excellent or good clinical results at the most recent follow-up. Conclusion: For advanced secondary coxarthrosis, total hip arthroplasty with use of a proximal modular femoral stem yielded good mid-term results based on clinical and radiological criteria.
Arthritis ; Arthroplasty, Replacement, Hip* ; Coxa Vara ; Diagnosis ; Female ; Femur* ; Follow-Up Studies ; Hip Dislocation ; Hip* ; Humans ; Osteoarthritis, Hip* ; Osteolysis ; Periprosthetic Fractures ; Postoperative Complications ; Sciatic Neuropathy

Primary cancer af the duodenum is rare. An extensive experience with fiberoptic eaophag-ogastro-duodenoscopy in our institution has indicated that duodenal cancer may be more frequent thsn suspeeted and readily diagnesed by this modality, With this in mied, clinical, pathological, diagnostic and therapeetie aspects of the 26 patients with primary duodenal cancer which had been endoscopically diugnosed were reviewed. The results obtained were as follows: 1) Ages ranged from 16 to 75, with the mean of 55.2 years. Seventeen patients were men and nine patients women being l. 9:1 of male-female ratio. 2) Hndoscoyic findings were 46. 1 percent ulcerative anld 34. 8 per cent ulcerative-infiltrative, 3) The diatribution within the duodenum was 50 per cent suprapapillary, 42. 4 per cent peri- papillary aecl 7. 6 per cent infrapapillary. 4) The twenty-three patients had adenocarcinoma, two patients leiomyosarcoma and 1 patient lymphoma. 5) Twenty patients were confirmed by duodenoseopic biipsies and six patients by operations. 6) The mast common presenting mmyliiints were abdominal pain, chronic blood lais leading anemia, jaundice, anorexia, indigestion, weight lass and palpable abdominal mass. 7) Of the 26 patients, eight patients underwent attempted curative resection, six patients underwent palliative bypasa procedures or exploratory laparotomy.
Abdominal Pain ; Adenocarcinoma ; Anemia ; Anorexia ; Diagnosis* ; Duodenal Neoplasms ; Duodenum ; Dyspepsia ; Female ; Humans ; Jaundice ; Laparotomy ; Leiomyosarcoma ; Lymphoma ; Male ; Ulcer

Postcholecystectomy syndrome is the persistence or recurrence of symytom complex following cholecystectomy, The majority of the patients have mild symptoms. However, the cause of recurrent symptoms is often obscure and as a consequence, a plan of management is difficult to formulate. Recently ERCP has proved to be increasingly helpful in the investigation of postcholecystectomy patients. The purpose of this study was to test the effectiveness of the ERCP in the evaluation of patients with postcbiolecystectomy syndrorae. The results are as follows 1) 102 postcholecystectomy patients were studied by endoscopic retrograde cholangiopancreatography and successful cannulation with demonstration of at least one duct was achieved in 49 of 50 jaundiced patients and in 49 of 52 non-jaundiced patients. The overall success rate was 96. 1%. 2) The results of ERCP were normal in 26 patients(26.5%) and abnormal in 72 patients (73.5%), Only 3 of 50 jaundiced patients were normal, but 23 of 52 non-jaundiced patients showed no abnormal findings. 3) The most common abnormality was biliary stone in common bile duct and intrahepatic duct which were present in 51 patients(50%). Cholangitis without stone was next common finding which was in 13 patients(13%). Of the remaining patients have air biligram, 2 CBD stricture, 1 CBD aseariasis & 1 chronic pancreatitis. 4) Time lapse between onset of symptoms and cholecystectomy was variable. 31 patients were studied less than 2 years after cholecystectomy. 18 of these patients had jaundice and 13 had no jaundice. Within 2 years afte chklecystectomy, the biliary stone was most common finding which were present in 14 out of 31 patients.
Catheterization ; Cholangiopancreatography, Endoscopic Retrograde* ; Cholangitis ; Cholecystectomy ; Common Bile Duct ; Constriction, Pathologic ; Humans ; Jaundice ; Pancreatitis, Chronic ; Postcholecystectomy Syndrome ; Recurrence

The majority of cystic lesions of the pancreas are psudocysts and a small fraction neoplastic. Failure to recognize the true nature of neoplastic cyst will lead to an incorrct treatment strategy. Ultrasonography, computerized tomography and angiography were used to distingish these lesions, but diagnostic value of ERCP is in controversy. To evaluate the diagnostic value of ERCP in cystic lesions of the pancreas, we analysed 33 cases of pancreatic cystic lesions (pseudocyst 18 cases, retention cyst 3 cases and cystic, neoplasm l2 cases) between Apr. 1985 and June 1993. In 18 cases of pseudocysts, ERP findings were communication with cyst in 8 cases (44.4%), chronic pancreatitis in 8 cases (44.4%), obstruction in 4 cases (22.2%) and displscement of pancreatic duct in 2 cases (11.1%), and ERC findings, which were perfomed in 8 cases, showed cholangitis in 3 cases (37.5%), CBD stone in 2 cases (25%), mass effect in 1 case (12.5%) and normal in 2 cases (25%). There was no communication with the cyst and pancreatic duct, except two mucinous ductal ectasia, in 12 cases of cystic neoplasms, and the other findings were displacement of pancreatic duct in 4 cases (33.3%), obstruction in 2 cases (16.7%) and normal in 4 cases (33.3%). ERC findings of cystic neoplasm were almost normal (85.7%) except 1 case of cholangitis. In conclusion, ERCP findings of pseudocysts were communication with pancreatic duct, chronic pancreatitis and biliary tract abnormality. In contrast, ERCP findings of cystic neoplasms were displacement or obstruction of pancreatic duct without communication and chronic pancreatitis, and biliary tract abnormality were rare.
Angiography ; Biliary Tract ; Cholangiopancreatography, Endoscopic Retrograde* ; Cholangitis ; Dilatation, Pathologic ; Mucins ; Pancreas ; Pancreatic Cyst* ; Pancreatic Ducts ; Pancreatitis, Chronic ; Ultrasonography

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

Malignant histiocytosis is a rare, usually fatal malignant neoplasm of reticuloendothelial systems. The disease is associated with fever, malaise, weight loss, hepatosplenomegaly, lymphadenopathy, pancytopenia, jaundice, and purpura. A 44-year-old female patient is described who had multiple, purple crusted nodules and plaques in the skin. In the laboratory study, pancytopenia was noted on the peripheral blood. In addition many atypical histiocytes were seen on the bone marrow aspiration. A lesional biopsy showed nodular infiltrations of atypical histiocytes in the dermis and some erythrophagocytosis was seen. Immunohistochemically, the histiocytes were weakly stained for lysozyme and α-l-antichymotrypsin, but were unstained for S-100 protein, cytokeratin, CEA(carcinoembryonic antigen), pan T/B marker CD30(ki-1), UCHL-1 LCA(leukocyte common antigen), and α-l-antitrypsin.
Adult ; Biopsy ; Bone Marrow ; Dermis ; Female ; Fever ; Histiocytes ; Histiocytic Sarcoma* ; Humans ; Jaundice ; Keratins ; Lymphatic Diseases ; Mononuclear Phagocyte System ; Muramidase ; Pancytopenia ; Purpura ; S100 Proteins ; Skin ; Weight Loss

PURPOSE: The purpose of this study is to survey health requirements of middle-aged women and their families and to provide guidelines for developing nursing interventions by describing the process of family health maintenance experienced by middle-aged women and its conceptual system. METHODS: To get saturated data, each of four researchers conducted two or three times of in-depth interview with eight middle-aged women aged between 40-64 years old and living in Seoul and Chuncheon from the 10th to the 30th of October 2004 and each interview was continued one or two hours. The Grounded theory adopted by Strauss & Corbin (1998) is a substantive theory that can explain the experiencing process of middle-aged women. RESULTS: We found that the casual condition of family health maintaining by middle-aged women was "confidence of health belief", and "pouring by body moving" was found to be its phenomenon. A textual condition that might respond to the phenomenon was "fatal roles acceptance", and intervening conditions that promote their family health were "retracing" and "gathering health information". These intervening conditions impacted middle-aged women's confidence in family health and led them to take actions/interactions such as "being a model of health", "adapting to circumstances", "do-it-myself", "taking-care" "harmonious mind" and "the pursuit of cleanness". These actions/interactions produced results such as "being stronger", "being unmanageable" and "being fruitful". CONCLUSIONS: Health confidence and practical health behaviors were observed in the process that the middle-aged female participants experienced unmanageable circumstances but they accepted their roles and responsibilities and recognized that they must be get stronger. The behaviors of health-together-with were divided into enthusiastic type, adaptation type and self-sacrificing type. Therefore middle-aged woman with the understanding of family health maintaining process as well as the theoretical system and practical principals needs to implement the intervention in acceptable level of family health process of preventing psychological and physical problems.
Family Health* ; Female ; Gangwon-do ; Health Behavior ; Humans ; Nursing ; Seoul